Audiology and hearing

Predictive value of early ABR testing in children with language/communication pathology

I. Aras, R. Drvis                                                                                                                                                    doi: 10.48095/ccorl2025S1_73

SUVAG Polyclinic, Zagreb, Croatia

Introduction: The connection between pathology of hearing and speech language pathology is widely known, but recognizing the sounds of speech and acquiring language involves neural structures far beyond the peripheral sensory organ of hearing. Auditory brainstem response (ABR) is an electrophysiological measure of a part of the hearing pathway that can give us some insight of the hearing function on a brainstem level. Aims: The aim of the study was to estimate the predictive value of ABR latencies testing in connection to language and communication pathology. Materials and Methods: The participants of the study were 50 very young children with nonspecific but clinically noticed language / communication delay, aged between 2 and 3,6 years. Only children with TG type A and normal peripheral hearing measured by ASSR were involved in the study. Supraliminal ABR testing was performed in usual conditions during sleep, and absolute and inter peak latencies of the waves I, III and V were observed. At the age of 4–5 years, children were tested with standardized language tests, tests of psychomotor development, and were examined by child psychiatrist and pediatric neurologist if needed. According to the findings, they were subdivided in several groups: 1. children with DLD; 2. children with psychomotor retardation; 3. children with autism spectrum disorder (ASD); and 4. children without significant language pathology. Statistical analysis examined the connection between prolongation of ABR latencies and type of pathology. Results: Preliminary results show that the prolongation of ABR latencies is mostly seen in group of children with ASD, less in DLD, and very rare in children with psychomotor retardation.

Endoscopic ear surgery – Why not be afraid and our initial experiences

M. Enter, R. Pacola, J. Syrovátka                                                                                                                     doi: 10.48095/ccorl2025S1_74

ENT and Head and Neck Surgery Department, Hospital AGEL Nový Jičín, Nový Jičín, Czech Republic

Introduction: Endoscopic ear surgery (EES) is a minimally invasive approach for treating middle ear pathologies, offering an alternative to classic microscopic surgery. We present our initial experiences and a series of cases using this method. Materials and Methods: We present a group of 11 patients who underwent transcanal endoscopic ear surgery for ear pathology, all performed during 2023 and 2024. The majority of cases involved chronic tympanic membrane perforation. Results: We performed 40 ear surgeries during 2023 and 2024, including 15 myringoplasties, 15 cholesteatoma surgeries, and 10 canalplasties. Of the myringoplasty surgeries, 66% (10) were performed using the total endoscopic approach, while 33% (5) were performed using the microscopic approach. EES myringoplasty was primarily used for dorsal tympanic membrane perforation (70%), and no cases required conversion to a microscopic approach. The microscopic approach was chosen primarily for anterior perforations (60%) or total perforations (40%). Only one endoscopic surgery was a second-look procedure. We encountered one complication: graft movement, which required revision surgery. Conclusion: Endoscopic ear surgery is a modern approach to treating middle ear pathologies, either as a totally endoscopic procedure or as an endoscopically assisted microscopic surgery, with favorable outcomes for patients. A thorough preoperative examination and careful patient selection are crucial for the success of this approach.

Malingering of one-sided hearing loss – a case report and review of audiological methods

J. Heřman, V. Glumbíková                                                                                                                                doi: 10.48095/ccorl2025S1_75

Department of Otorhinolaryngology and Head and Neck Surgery, Palacký University Olomouc and University Hospital Olomouc, Olomouc, Czech Republic

We present a case of 19-year-old patient malingering gradual unilateral hearing loss. We also review audiological methods applicable to detect such simulation.

Hearing screening in adults over 50 in the Czech Republic: preliminary data from a pilot study

M. Homoláč1, V. Chrobok1, J. Dršata1, J. Krtičková1, K. Hejduk2,3, L. Mandelová2, R. Chloupková2,3            doi: 10.48095/ccorl2025S1_76

1 Department of Otorhinolaryngology and Head and Neck Surgery, Charles University, Faculty of Medicine in Hradec Králové and University Hospital Hradec Králové, Hradec Králové, Czech Republic

2 National Screening Centre, Institute of Health Information and Statistics of the Czech Republic, Prague, Czech Republic

3 Institute of Biostatistics and Analyses, Faculty of Medicine, Masaryk University, Brno, Czech Republic

Introduction: Hearing impairment of some degree is a prevalent condition among older adults, affecting an estimated 65% of individuals above 60 years of age, according to the World Health Organization, and often leading to social isolation and cognitive decline. An effective nationwide screening program could significantly increase the chances of early detection, enabling timely interventions to prevent further deterioration of hearing. Aims: To assess the feasibility and effectiveness of different hearing screening methods in adults aged 50+, identify the most effective methods for use in primary care, and provide insights for developing a national screening program in the Czech Republic. Materials and Methods: This two-centered single-arm quasi-experimental study aims to enroll 330 participants aged 50 years or older. Screening is conducted in two steps, first by general practitioners using three methods: 1. an abbreviated version of the Hearing handicap inventory for adults (HHIA) questionnaire; 2. a whispered voice test; and 3. screening pure-tone audiometry at 35 dB at four frequencies (500; 1,000; 2,000; 4,000 Hz). In second step, participants undergo confirmatory testing by ENT specialists, including otoscopy, tympanometry and pure-tone audiometry, which serve as the reference standard. Results: Preliminary analysis will evaluate the feasibility of integrating these methods into primary care and their effectiveness in detecting hearing loss. Expected findings include concordance rates between screening tools and the reference standard, aiding in determining optimal methods. Conclusion: This study investigates integrating hearing screening into primary care for adults aged 50+. Preliminary data will inform the development of a standardized screening program, enhancing early detection and management of hearing loss in the aging population of the Czech Republic.

Supported by the Cooperatio Program, research areas SURG and DIAG, and the project Data, Analytical, and Information Support for Quality Improvement of Early Detection Programmes (CZ.03.02.02/00/22_005/0000283).

The effect of balance belt on sensory organization test in individuals without balance disorders

R. Hürriyetoğlu1, O. Yılmaz2, C. Yeral2                                                                                                                                                                   doi: 10.48095/ccorl2025S1_77

1 Department of Audiology, Ankara Medipol University, Ankara, Türkiye

2 Department of Audiology, İstanbul Medipol University, İstanbul, Türkiye

Introduction: The vestibular system integrates visual, proprioceptive, and vestibular inputs to maintain postural stability. Balance disorders disrupt daily activities, necessitating rehabilitation methods like vibrotactile feedback. The BalanceBelt is a wearable device designed to enhance balance through vibrotactile stimulation. This study evaluates the BalanceBelt’s effect on sensory organization in individuals without balance disorders. Aims: This study aims to determine if the BalanceBelt enhances somatosensory input and improves balance control in healthy individuals. Postural stability was assessed across different Sensory Organization Test (SOT) conditions with and without the BalanceBelt. Materials and Methods: Thirty healthy participants (19–29 years) underwent the SOT twice: once without and once with the BalanceBelt. The BalanceBelt (Elitac Wearables, Netherlands) provided vibrotactile stimulation. The NeuroCom system assessed postural stability across six conditions. Wilcoxon Signed-Rank Test analyzed equilibrium scores. Results: Significant improvements were found in conditions C3 and C4 (P < 0.05) and in the SOM1 score (P < 0.05) with the BalanceBelt, suggesting enhanced somatosensory input. No significant differences were noted in composite equilibrium scores or other conditions (P > 0.05). Conclusion: The BalanceBelt enhances somatosensory input in specific postural conditions but does not significantly improve overall balance in individuals without balance disorders. Future research should explore its application in vestibular rehabilitation for patients with balance impairments. The BalanceBelt offers potential as a non-invasive tool for postural control in bilateral vestibular dysfunction.

Impact of endolymphatic sac decompression on hearing function in patients with Menière’s disease

L. Murgašová, K. Bubáková, M. Stříteská, K. Trnková, Ľ. Verešpejová, Z. Urbániová, D. Čapková, M. Chovanec                                                                                                                                 doi: 10.48095/ccorl2025S1_78

Department of Otorhinolaryngology, Charles University, 3rd Faculty of Medicine and University Hospital Královské Vinohrady, Prague, Czech Republic

Introduction: Menière’s disease (MM) is a paroxysmal vestibular disorder caused by idiopathic endolymphatic hydrops. Treatment is symptomatic, with medication as first-line therapy. When conservative treatment fails, surgical options such as endolymphatic sac decompression (ELSD) are considered for intractable vertigo. ELSD is an effective stabilising surgical option, though it carries the risk of postoperative auditory-vestibular dysfunction. Aims: This study aims to evaluate the impact of ELSD on hearing. Materials and Methods: We retrospectively analyzed 37 patients (25 women, 12 men; mean age 50) who underwent ELSD for intractable vertigo due to definitive MM at our institution between 2019 and 2023. A total of 38 decompressions were performed, including one bilateral procedure. Hearing was assessed using pure-tone audiometry. Pure tone average (PTA) was calculated from frequencies of 500; 1,000; 2,000; and 4,000 Hz for both bone and air conduction. Hearing changes were evaluated by comparing preoperative and 12-month postoperative audiometry, with a ≥ 5 dB change in PTA considered significant. The emergence of a low-frequency air-bone gap (LFABG; ≥10 dB at 250; 500; 1,000 Hz) was also analyzed. Hearing difficulties were evaluated using the Hearing handicap inventory (HHI). Results: Postoperatively, bone conduction thresholds deteriorated in 53.1% of patients, remained unchanged in 28.1%, and improved in 18.8%. Low-frequency thresholds generally improved, while those at 2,000, and 4,000 Hz worsened. The mean PTA for bone conduction increased from 30.7 to 39.3 dB postoperatively, while air conduction PTA increased from 36 to 49.2 dB. LFABG developed in 53%. HHI scores showed no significant changes between preoperative and postoperative assessments, remaining within the moderate deficit range. Conclusion: ELSD is effective in stabilising vertigo in MM. Postoperative complications are rare. Even though it carries the risk of worsening bone and air conduction thresholds, ELSD offers minimal morbidity with better preservation of auditory and vestibular function compared to ablation techniques.

Levels of peripheral myelin protein and vitamin D in the blood serum of patients with type 2 diabetes and hearing impairments

N. Navalkivska, T. Shydlovska                                                                                                                          doi: 10.48095/ccorl2025S1_79

Voice and Hearing Department, State Institution “O.S. Kolomiychenko Institute of Otolaryngology of National Academy of Medical Science of Ukraine”, Kyiv, Ukraine

Introduction: Patients with diabetes mellitus often experience microand macrovascular complications requiring early diagnosis and treatment. Metabolic dysfunction and disorders of the hemostatic system are key factors in the development of complications in type 2 diabetes mellitus, including degenerative changes in nerve fibers. Hearing impairments, such as sensorineural hearing loss (SNHL), are commonly observed in diabetes. Changes in metabolism and vitamin deficiencies also contribute to the development of SNHL. An important aspect of treatment is timely identification of demyelinating processes and assessment of vitamin D levels in blood serum. Aim: The aim of the study was to examine the levels of peripheral myelin protein 22 (PMP 22) and vitamin D in patients with type 2 diabetes and hearing impairment. Materials and Methods: 43 patients with type 2 DM (21 men, 22 women, aged 56.28 ± 4.54 years) with degree I–II sensorineural disorders, 15 individuals with SNHL (8 men, 7 women, aged 59.33 ± 2.65 years) without diabetes, and 15 controls were examined. The patients were divided into two groups: the 1st group (N = 21) with or without isolated microvascular complications of type 2 DM, and the 2nd group (N = 22) with neuro-, nephro-, and retinopathy. All patients underwent laboratory tests, clinical and instrumental assessments of auditory function. The obtained data were analyzed statistically. Results: The analysis revealed that in both groups with type 2 DM and SNHL, PMP22 levels were significantly higher than in the controls, especially in those with microvascular complications. Vitamin D levels were significantly lower in both groups with type 2 DM and SNHL compared to controls, with a more pronounced decrease in the group with complications. Conclusion: Our findings suggest that demyelinating processes and vitamin D deficiency may play a crucial role in sensorineural hearing loss in type 2 diabetes patients.

Preoperative frequency allocation accuracy in Cochlear implantation: the impact of Cochlear duct length on electrode positioning

D. Paouris1, L. Varga2                                                                                                                                                                                                               doi: 10.48095/ccorl2025S1_80

1 Department of Otorhinolaryngology, Head and Neck Surgery, Comenius University and the University Hospital in Bratislava, Bratislava, Slovakia

2 Department of Otorhinolaryngology, Head and Neck Surgery, Comenius University and the University Hospital in Bratislava, Biomedical Research Center SAS, Bratislava, Slovakia

Introduction: Accurate preoperative frequency allocation prediction is crucial for optimal cochlear implant outcomes. Aims: To evaluate the accuracy of preoperative frequency allocation predictions and their relationship with cochlear duct length (CDL) in cochlear implantation. Materials and Methods: This retrospective observational study analyzed 50 implanted ears. Preoperative frequency allocations for 12 electrodes were estimated using OTOPLAN software and compared with postoperative measurements. CDL was measured for each patient. Frequency-to-place mismatch and angular insertion depth (AID) were calculated and analyzed in relation to CDL using paired t-tests and Spearman’s correlation. Results: Mean CDL was 35.6 mm (SD 1.68 mm). Significant differences were found between preoperative and postoperative frequency allocations for electrodes C11 to C1 (all P < 0.001). The mean preoperative AID estimation (576°, SD 57.3°) significantly overestimated the postoperative measurement (531°, SD 94.2°F; P = 0.002). Frequency-to-place mismatch ranged from 4374 Hz (C12) to 112 Hz (C1), showing a consistent overestimation of frequencies. A moderate negative correlation was observed between CDL and frequency-to-place mismatch (Spearman’s rho = –0.5315; P = 0.0754), suggesting that longer cochleae tend to have smaller mismatches, although not statistically significant at the 0.05 level. Conclusion: Preoperative frequency allocation predictions using OTOPLAN consistently overestimate postoperative measurements, with the degree of mismatch potentially influenced by CDL. This study highlights the importance of considering individual cochlear anatomy in preoperative planning for cochlear implantation. Further research is needed to refine prediction models and improve the accuracy of preoperative frequency allocation estimates.

AirPods Pro 2 as a game changer in hearing rehabilitation? A pilot study on mild to moderate hearing loss and tinnitus

M. Ševčík                                                                                                                                                              doi: 10.48095/ccorl2025S1_81

ENT, Třinec Hospital, Třinec, Czech Republic

Introduction: Conventional hearing aids remain the gold standard for hearing rehabilitation, but consumer devices like AirPods Pro 2, featuring built-in audiometry and adaptive transparency, may offer a viable alternative for individuals with mild to moderate hearing loss or tinnitus. Aims: This study evaluates the effectiveness of AirPods Pro 2 compared to conventional hearing aids, focusing on speech intelligibility, tinnitus relief, and user comfort. Materials and Methods: A pilot study is being conducted on adult patients with mild to moderate sensorineural hearing loss and/or tinnitus. Each participant undergoes a comprehensive audiometric assessment, followed by a comparative trial using AirPods Pro 2 and a professionally fitted hearing aid deemed optimal for their condition. The primary outcome measures include speech-in-noise performance, tinnitus relief (measured via subjective scales), and user-reported comfort and satisfaction. Results: Preliminary findings suggest that AirPods Pro 2 improve speech intelligibility in quiet and moderate noise. Some patients report tinnitus relief with adaptive transparency mode. While hearing aids generally perform better in complex environments, AirPods Pro 2 present a promising alternative. Conclusion: Our findings suggest that consumer-grade devices like AirPods Pro 2 should be considered in hearing rehabilitation strategies, particularly for individuals with mild to moderate hearing loss who seek an affordable and user-friendly option. Further research is needed to refine their clinical application and determine their long-term benefits compared to conventional hearing aids.

Speech understanding with ISS masking in patients with single-sided Cochlear implantation

Ľ. Šuchová                                                                                                                                                            doi: 10.48095/ccorl2025S1_82

Phoniatric Department, University Hospital Bratislava, Bratislava, Slovakia

Introduction and Aims: In our group, we tested 47 patients after unilateral cochlear implantation without using a hearing aid on the non-implanted ear and 1 patient after bilateral cochlear implantation. For testing, we used Slovak speech audiometry SSA at an intensity level of 60 dB with an ISS signal at an intensity level of 50 dB. During testing, the patient had to repeat a decade of SSA words from a distance of 0.5 m, first from the side without the implant, then from the side with the implant. The aim was to confirm the fact that speech understanding in background speech noise is better on the side of the cochlear implant. Materials and Methods: Number of patients was 48, men 22, women 26, average age was 46.5, minimum 18, maximum 78 years. Sides of implantation – right side 30, left 17, both sides 1 patient. Average age at the time of surgery was 36.52 years, minimum 1.5, and maximum 77 years. Number of years since KI was on average 10.02 years, minimum 0.25, and maximum 28 years. Implant type was Medel-33, Nucleus-15. Cause of hearing impairment was unknown in 28 patients, genetics impairment 10, after meningitis 3, after noise exposure 4, after fracture of the osis temp.1, after application of STM 1, after stroke 1. Results: The number of repeated words from the side with a speech processor was on average 84%, minimum 40%, and maximum 100%. From the side without a speech processor was on average 63%, minimum 30%, and maximum 90%. In patient with two speech processors the number of repeated words from the right side was 100%, from the left side 90%. Conclusion: All patients after unilateral cochlear implantation achieved a better result when repeating words from the side of the cochlear implant.

Vestibular profile of patients with hearing loss caused by pathogenic variants of the STRC gene

V. Svobodová1, Z. Balatková1, Z. Libaková1, A. Procházková1, D. Šafka Brožková2, M. Komarc3,

V. Koucký4, M. Bonaventurová4, V. Kruntorád5, Z. Čada1                                                                                                              doi: 10.48095/ccorl2025S1_83 

1 Department of Otorhinolaryngology, 2nd Faculty of Medicine, Charles University in Prague and University Hospital Motol, Prague, Czech Republic

2 DNA Laboratory, Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic

3 Department of Anthropomotorics and Methodology, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic

4 Department of Otorhinolaryngology and Head and Neck Surgery, 1st Faculty of Medicine, Charles University, and Motol University Hospital, Prague, Czech Republic

5 Department of Pediatric ENT, University Hospital Brno, Brno, Czech Republic

The second most frequent cause of non-syndromic autosomal recessive hearing loss (AR-NSHL) worldwide is a biallelic pathogenic alteration of the STRC (stereocilin) gene, also named DFNB16. The type and severity of hearing impairment in DFNB16 patients were studied thoroughly, while information on a detailed examination of their vestibular function is still lacking. Our aim was to characterize the vestibular status of patients with biallelic pathogenic variants in STRC by performing a complete up-to-date test battery. Eight AR-NSHL patients, aged 6–37 (mean age ± SEM 16.13 ± 8.67), underwent standard audiological testing and otoneurologic investigation including videonystagmography (VNG) with caloric stimulation, video head-impulse test (vHIT), and cervical vestibular evoked myogenic potentials (cVEMPs). Subjects were divided into three groups (group 1, 2, 3) according to the type of diagnosed STRC gene variant. The grade of the hearing loss was calculated as PTA (mean PTA ± SEM 41.88 dB ± 5.49). The vHIT displayed nearly normal bilateral gain and mostly the absence of saccades in all examined groups. Cervical VEMPs in response to AC and BC stimuli showed prolonged latencies of waves P1 and N1 bilaterally in group 1, although latencies in group 2 and 3 were within normal range. The results of VNG indicated normal vestibular and central oculomotor function. Biallelic pathogenic variants in STRC gene influence inner ear’s cochlear and vestibular function. Vestibular dysfunction in DFNB16 patients was detected by detailed evaluation, despite none of the DFNB16 subjects in our study reported subjective symptoms.

SmartNav in cochlear implantation under local anesthesia

M. Tesařová, P. Kalitová, S. Cha, M. Vojtová, J. Plzák, J. Bouček                                                           doi: 10.48095/ccorl2025S1_84

Department of Otorhinolaryngology and Head and Neck Surgery, 1st Faculty of Medicine, Charles University, and Motol University Hospital, Prague, Czech Republic

Cochlear implantation is an effective and safe method of rehabilitation for severe hearing loss, regardless of the age of the patient. Particularly in older patients, the risks associated with general anaesthesia are increasing and we must consider whether they are acceptable in the pursuit of improved quality of life. In recent years, cochlear implantation has been increasingly performed under local anaesthesia (LA) or in combination with analgosedation (AS). SmartNav is a new technology that enables continuous monitoring of electrode array insertion in Cochlear implants, determination of angular depth of insertion, performance of transimpedance matrix (TIM) and exclusion of tip-fold over, measurement of impedances and standard neural response telemetry (NRT). In the present cohort, we used it in senior patients operated with a combination of LA and AS. Between January and April 2024, we performed the study at the Department of Otorhinolaryngology Head and Neck Surgery, 1st Faculty of Medicine, Charles University, University Hospital Motol, Prague and performed CI under the combination of LA and AS in a total of 11 patients in whom electrode array insertion was monitored by SmartNav. The average age of the patients was 76.2 years, all were male, and we operated four times on the left and seven times on the right ear. All patients had progressive hearing loss in adulthood. Full insertion of the CI622 electrode array was achieved in all patients. The average angular depth of insertion was 332 degrees, the average insertion velocity was 0.32 mm/s. TIM excluded tip-fold over in all patients, which was subsequently confirmed by postoperative X-ray. In 5 of the 6 patients, the procedure could be completed under AS/LA, in one patient the procedure had to be converted and completed under general anesthesia, using a laryngeal mask. Cochlear implantation under LA/AS is a safe method to minimize the risks associated with general anesthesia. All SmartNav system features could be used during surgery, with the exception of NRT. In the presented miniseries, there was 100% concordance of the TIM excluding tip-fold over and postoperative X-ray. The SmartNav system is an effective and safe tool even for procedures performed in LA or AS.

Funding: NW24J-06-00119, GAUK 194423.

Sound therapy and tinnitus retraining therapy in patients with chronic subjective tinnitus

Z. Veldová1, K. Janků2                                                                                                                                                                                                             doi: 10.48095/ccorl2025S1_85

1 Department of Otorhinolaryngology and Maxillofacial Surgery, Military University Hospital, Prague, Czech Republic

2 Sleep Disorders Clinic, National Institute of Mental Health, Klecany, Czech Republic

Introduction: The work examines the impact of chronic subjective tinnitus on the personal and professional lives of patients, focusing on its effect on their mental health (anxiety, depression, difficulty coping with stress and emotions) speech comprehension disorders and sleep quality. The increasing prevalence of tinnitus patients highlights the importance of a multifactorial therapeutic approach, in which sound therapy using TRT (Tinnitus retraining therapy) plays a crucial role. Aims: To present the effect of sound therapy (pink noise) within the framework of TRT using TCI (Tinnitus control instruments) – a noise generator – on improving the quality of life of patients with chronic subjective tinnitus. Material and Methods: A cohort of 106 patients with chronic subjective tinnitus was selected. Patients underwent neurological examination, including brain MRI. Before the application of TCI (always binaurally), the following parameters were assessed: hearing threshold, tinnitus frequency characteristic and intensity, uncomfortable loudness level threshold and speech audiometry in a free-field setting. Patients completed the THI (Tinnitus handicap inventory) questionnaire. Pink noise in the TCI was adjusted to match the frequency characteristics of the patients’ tinnitus, with an intensity lower than the perceived tinnitus. Comprehensive audiometric examinations and THI questionnaires were evaluated after 12 and 24 months. Results: Both the pitch and intensity of tinnitus decreased over 12 months. The number of points in the THI questionnaires gradually reduced after 12 and 24 months of treatment. These results correlate with a reduction in negative impacts on patient ‚s lives, including improved sleep quality. Discussion and Conclusion: Our findings demonstrate that TRT, within the framework of CBT (Cognitive behavioral therapy) has a significant role in the treatment of tinnitus.

Permanent conductive hearing loss in children: etiology, typical audiograms and treatment

S. I. Zabaneh1, L. E. Hahn1, M. Fleischer1, J. Althaus2, F. Wohlfarth1, C. Männel1, D. Mürbe1, A. Hirschfelder1                                                                                                                                                                                             doi: 10.48095/ccorl2025S1_86

1 Klinik für Audiologie und Phoniatrie, Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany

2 Klinik für Ohren-, Nasen-, Halsund Gesichtschirurgie, Universitätsspital Zürich, Berlin, Germany

Introduction and Aims: Permanent conductive hearing loss (CHL) in children often presents alongside comorbidities, complicating timely diagnosis. This study aims to explore the etiology, audiologic profiles, and treatment of affected children. Materials and Methods: Data from 1,043 children with CHL were collected from the German Registry for Hearing Loss in Children. Analysis considered reported comorbidities, risk factors, degree of hearing loss according to WHO 2021, audiogram types, treatment, and age at diagnosis. Audiogram classification (N = 789 ears) was conducted using shape-similarity and minimum variance to map standard audiograms. Results: CHL was reported in 6% of children in the registry, with bilateral CHL in 52% of affected children. Comorbidities in CHL (CHL+) in contrast to isolated CHL were observed in 85% of the cases, predominantly craniofacial malformations (79%). Risk factors were reported in 22%, with need of ventilation (4.3%), parental consanguinity (4.2%), prematurity (3.8%), and hypoxia (3.5%) being the most common risk factors. Audiogram analysis revealed pantonal hearing loss in half of the cases as well as low-frequency and tent-shaped losses being more prevalent than high-frequency loss. Degrees of hearing loss ranged from mild (13%) to severe (19%). Median diagnostic age for CHL+ children was 0.7 years (IQR 0.2–4.1 years), while isolated CHL was diagnosed later, at a median of 5.5 years (IQR 2.8–6.11 years). Air-conduction hearing aids were the most common type of treatment (79%) while bone-conduction aids were reported in 4.4% of the cases. Conclusion: In comparison to isolated cases, CHL was more commonly associated with comorbidities and these children were diagnosed earlier. Air-conduction hearing aids were the primary treatment. Pantonal balanced audiograms dominated, while high-frequency loss was rare. Thus, early frequency-specific bone-conduction threshold assessments (e. g., BERA) are further recommended, especially for atypical audiograms.