Journal of Otorhinolaryngology, Hearing and Balance Medicine

The universal newborn hearing screening (NHS) program was implemented in Russia in 2008 to replace the high-risk newborn hearing screening. More than 95% coverage and significant improvement in early detection and intervention is achieved. Meanwhile, it was shown that current OAE-based hearing screening missed 13% of newborns with genetically ascertained hereditary sensorineural hearing loss (SNHL). The aim of the study is to assess the results of genetic investigation and NHS in a large cohort of Russian children with bilateral SNHL and to study the feasibility of implementation of combined hearing and genetic screening in Russia. Genetic, audiological and NHS data of 1292 pediatric patients with bilateral SNHL born in 2008–2021 were analyzed. GJB2 sequencing was performed for all subjects, 644 patients had pathological GJB2 genotype, 406 of them were homozygous for c.35delG variant. The group of 155 GJB2-negative patients were searched for other SNHL genes, The pathological genotypes were identified at 87 patients. The most frequent genes were STRC (21.8%), USH2A (16.1%), OTOF (8%) and SLC26A4 (6.9%). Children with confirmed genetic etiology passed NHS in 21% of cases. The perspectives of implementation of national comprehensive newborn hearing and genetic screening including whole exome sequencing technologies are discussed. Full article

Speech intelligibility is a concern for public health, especially in non-ideal listening conditions where listeners often listen to the target speech in the presence of background noise. With advances in technology, synthetic speech has been increasingly used in lieu of actual human voices in human–machine interfaces, such as public announcement systems, answering machines, virtual personal assistants, and GPS, to interact with users. However, previous studies showed that speech generated by computer speech synthesizers was often intrinsically less natural and intelligible than natural speech produced by human speakers. In terms of noise, listening to synthetic speech is challenging for listeners with normal hearing (NH), not to mention for hearing-impaired (HI) listeners. Recent developments in speech synthesis have significantly improved the naturalness of synthetic speech. In this study, the intelligibility of speech generated by commercial synthesizers from Google, Amazon, and Microsoft was evaluated by both NH and HI listeners in different noise conditions. Compared to a natural female voice as the baseline, listeners’ listening performance suggested that some of the synthetic speech was significantly more intelligible even at rather adverse listening conditions for the NH cohort. Further acoustical analyses revealed that elongated vowel sounds and reduced spectral tilt were primarily responsible for improved intelligibility for NH, but not for HI due to their impairment at high frequencies and possible cognitive decline associated with aging. Full article

This study aimed to compare the diagnostic efficacy of the Halmágyi–Curthoys head impulse (thrust) test and Romberg’s test in detecting vestibular hypofunctioning among two groups of 50 vertigo patients each; the two groups were randomly assigned. The assessment utilized the visual analog scale (VAS) to quantify subjective experiences of vertigo. The results revealed distinctive patterns in the detection of vestibular hypofunctioning, highlighting the strengths and limitations of each test. The Halmágyi–Curthoys head impulse test demonstrated utility in identifying vestibular hypofunctioning and its effect on vestibulo–ocular reflexes, particularly in cases with sudden head movements. Romberg’s test was useful in assessing postural instability in vestibular hypofunctioning due to defects in vestibulospinal reflexes. The integration of VAS scores provided valuable subjective insights into the patient experience. This comparative analysis contributes to a nuanced understanding of diagnostic tools for vestibular hypofunctioning in vertigo patients, offering clinicians valuable information for tailored assessments and interventions. Full article

Background: Body image is a potential psychological burden after total laryngectomy (TL) with devastating effects on patients’ health-related quality of life (HRQOL) and communication. This study focused on TL patients to determine the prevalence of dissatisfied body image and whether they have poorer HRQOL and difficulty adjusting to their new voice than TL patients with satisfied body image. It also aimed to investigate the potential predictors of body image. Methods: A multicenter cross-sectional study was conducted. For TL patients, the Body Image Scale (BIS), the European Organization for Research on Cancer Quality of Life Questionnaire, Core and Neck Module (EORTC QLQ C30 and EORCT H&N35), and the Self-Experiences of Communication after Laryngeal cancer (SECEL) were used. Patients were categorized as dissatisfied with their body image if the BIS score was ≥8. Multiple regression analysis was performed using the BIS as the dependent measure and HRQOL (QLQ C30 and H& N35) and communication (SECEL) as independent variables. Results: Overall, 31.3% of TL patients had dissatisfied body image, significantly worse HRQOL, and difficulty adjusting to their new voice than patients with satisfied body image. The regression model showed that social eating and socializing (H&N35) and adjustment to their new voice (SECEL) were independent predictors of body image. The model explained 52% of the variance. Conclusions: Screening TL patients at risk for body image concerns may help develop effective interventions to optimize HRQOL and patient communication. Full article

Implantation of two electrical stimulators of different cranial nerves in one patient is rare. We report the case of a forty-seven-year-old patient already implanted with a trigeminus nerve stimulator. In addition, this patient suffered from hearing problems. In one ear, the patient was deaf. On the other side, the patient wore a bone conduction hearing aid to improve hearing. In this complex situation, we decided to check the possibility of cochlear implantation on the deaf side. Finally, we managed to provide electrical stimulation of the auditory pathway of the deaf ear to improve the patient’s hearing tests. In addition, this case report shows how the trigeminus stimulator interferes with the electrical stimulation in auditory evoked potentials measurement of the auditory brainstem and cortex via EABR (evoked auditory brainstem response) resp. EALR (evoked auditory late response). Full article

Even if the management of parotid gland tumors depends on the histopathological subtype, preoperative imaging of parotid gland tumors is clinically relevant. Preoperative imaging gives insight into the differentiation between benign and malignant tumors, which might potentially decrease the number of unnecessary aggressive surgeries. Characteristic imaging findings on cross-sectional imaging, such as computed tomography (CT) and magnetic resonance imaging (MRI), can help narrow the differential diagnosis and guide the further management of patients presenting with parotid masses. While MRI is imperative for the determination of perineural spread, which is frequently encountered with malignant parotid tumors, CT is important for the evaluation of osseous invasion. Furthermore, multi-parametric MRI protocols provide insights into the tumor behavior and internal composition, which is helpful in the case of benign mixed tumors and others. While distant metastasis is uncommon with parotid neoplasms, PET/CT provides a valuable tool for the improved evaluation of loco-regional and distant metastatic disease. This article discusses the imaging features of common benign and malignant parotid tumors. Full article

Objective—The objective of this study was to systematically assess meta-analyses to determine the lacunae in the literature for PCF following laryngectomy. Methods—Bibliometric analysis were carried out on meta-analyses on PCF after total laryngectomy for laryngeal cancer in the PubMed database. Results—Twenty-four meta-analyses were considered eligible and chosen for analysis. Six meta-analyses (25%) focused on the risk factors for PCF in TL. Four meta-analyses (16.6%) focused on the role of the onlay flap. Four meta-analyses (16.6%) focused on the timing of feed initiation. Three meta-analyses (12.5%) focused on using a stapler for pharyngeal closure. Two meta-analyses focused on types of pharyngeal reconstruction. Other meta-analyses analyzed the use of salivary bypass tubes, the method of pharyngeal closure, organ preservation protocols on PCF, primary and secondary TEP, and the effect of non-surgical treatment on PCF. Conclusion—Despite plenty of published meta-analyses, there is a lack of scrutiny on certain critical aspects of PCF. Full article

Background: En block resection of squamous cell carcinoma (SCC) of the concha represents a reconstruction challenge, due to the complex topography and difficult access. Objective: The objective of the present paper is to describe the chemically assisted dissection (CADISS) of SCC originating in the auricular concha and the following reconstruction of the conchal cavity with a post-auricular island flap (PIF), taking care to minimize injury to the donor site. Methods: Twenty-six patients having a diagnosis of SCC of the auricular concha were included in the study. ‘En bloc’ removal of the tumor was accomplished, leaving the adjacent conchal cartilage attached to the tumor and using the CADISS technique to preserve the deep perichondrium. A PIF was used to repair the auricular conchal defect. Results: Flaps were normal at 10 days and at 1-month follow-up. No tumor recurrence was observed. No complications were observed. According to the SCAR scale, good aesthetic outcomes were achieved in all cases, both at the auricular concha and at the donor site. Conclusion: CADISS facilitates the complete removal of the tumor with the preservation of the surrounding normal tissues. A post-auricular island flap can be easily pulled through a post-auricular tunnel to repair the defect and the donor site can be closed primarily. Full article

Despite the evolution of hearing aids and cochlear implants, noisy environments are reportedly still an important hurdle for persons with hearing loss, especially in the process of speech recognition. The development of pre-processing algorithms and the pairing with a wireless device can bring relief to this situation, but it is still under scrutiny whether one or the other is more effective. The purpose of this study was to compare the benefits of speech recognition in a noisy environment by recipients of cochlear implants when using the pre-processing automatic algorithms or when using a wireless microphone. Twenty-nine participants were selected, aged 14 to 83, suffering from sensorineural hearing loss and recipients of cochlear implants for at least 6 months. The proprietary Cochlear Limited SCAN technology uses pre-processing algorithms to attenuate various noises; the wireless device MiniMic2 uses a 2.4 GHz connection to facilitate communications between the recipient and the signal source. Participants were asked to repeat 20 sentences randomly generated by the adaptive Italian Matrix Sentence Test, first while using the SCAN technology and then with the wireless MiniMic2. Both signal and noise were administered through a single loudspeaker set 1 m away from the subject. Significantly better results in speech recognition of noise were achieved with the wireless MiniMic2 when compared to the SCAN technology. Full article

(1) Background: Vocal fold nodules are bilateral lesions that can have an important negative impact on a person’s job performance, social interaction, and quality of life. This study aims to analyze multidimensional voice evaluation outcomes in a group of patients with bilateral vocal fold nodules who underwent voice therapy. (2) Methods: A retrospective analysis was performed in 42 patients on the following voice evaluations, before and after voice therapy: visual-perceptual (video-laryngostroboscopic evaluation), auditory-perceptual voice analysis based on the GRBAS scale, and aerodynamic voice analysis. Data were collected from January 2001 to December 2019. Data analyses were performed with non-parametric tests (Wilcoxon test) using α = 0.05. (3) Results: The patient average age was 33.6.1 ± 10 years (range 19–60), and 95.2% were female. Voice therapy was delivered by an experienced speech-language pathologist once a week, with an average of 9.8 ± 3 appointments (range 8–17). Vocal fold lesions disappeared in 40.4% of the patients after voice therapy, especially in participants receiving early voice therapy (p = 0.035). When comparing pre- and post-therapy audio-perceptual results, all parameters were improved with statistical significance (p < 0.05) except for the asthenic voice scale. Aerodynamic parameters were all improved but without statistical significance (p > 0.05); (4) Conclusions: Early timing to initiate voice therapy after the onset of symptoms or diagnosis seems to be an important factor for the success of voice therapy (absence of vocal fold nodules). Full article

Past studies have reported that there are higher sound-pressure levels for each vowel in a child’s ear canal than those in adults due to reduced volume and a shorter ear canal. Furthermore, longer vocal tracts are associated with lower formant frequencies, and vice versa. The structural differences in this regard may reflect the difference in the occlusion effect. Thus, the present study compares the sound pressure levels (SPLs) and first formant frequencies of children and adults with normal hearing and determines the best vowel stimulus to assess the occlusion effect. A repeated measures research design was utilized to investigate the best stimulus with which to measure the occlusion effect among children and adults. Group 1 included ten children, and Group 2 comprised ten adults with normal hearing. The SPLs at frequencies between 200 Hz and 1000 Hz for three uttered vowels, with steps of 100 Hz, were measured using a hearing aid analyzer. The recorded vowels were saved in a ‘.wav’ format for formant frequency analysis. Furthermore, a paired comparison method was used to identify the vowel stimulus that most effectively induced the occlusion effect. A significantly higher SPL was observed for children compared to adults for each vowel. The formant frequency F1 value was higher for children than adults for each vowel, constituting a significant finding. In the paired comparison, the occlusion effect was reported to be significantly greater with respect to the vowel /u/ among adults, while it was reported to be greater in relation to the vowel /i/ among children. The vowel /u/ was the best stimulus with which to assess the occlusion effect among adults. The vowel /i/ was the best stimulus with which to assess the occlusion effect among children. Full article

The Auditory Steady-State Response (ASSR) provides objective and ear-specific information essential for early and appropriate intervention. Test–retest reliability is essential for audiological monitoring. The test–retest reliability of the ASSR has received limited attention. Only a handful of studies found in the literature investigated the test–retest reliability of old-generation ASSR using amplitude or mixed modulated stimuli. However, to our knowledge, no published reports have specifically examined the test–retest reliability of the next-generation ASSR using Chirp family stimuli as implemented in the Interacoustics Eclipse system. This pilot study investigated (a) the test–retest reliability of air conduction (AC) ASSR thresholds across two test sessions and (b) the relationship between differences in ASSR thresholds across two sessions to the residual noise levels in normal-hearing adults. Methods: Fifteen normal-hearing adults (12 females) (30 ears) with an average age of 28 years were recruited for the study. The ASSRs were recorded using a two-channel recording montage. The automatic default stimuli and recording protocol using 90 Hz ASSR, and the accuracy method (p < 0.01) as implemented in the Eclipse system is used to measure ASSR. Results: The study demonstrated strong test–retest reliability for ASSR across frequencies (500 Hz, 1000 Hz, 2000 Hz, and 4000 Hz). Notably, the highest reliability was observed at 500 Hz. The mean test–retest reliability of ASSR was found to be comparable to pure-tone thresholds, but the intra-subject variability is higher for ASSR compared to pure-tone thresholds. Additionally, no significant correlation was found between the difference in ASSR residual noise levels at the threshold and the difference in ASSR thresholds at all tested frequencies. Conclusion: The next-generation system demonstrated strong test–retest reliability across the frequencies examined in this pilot study. Particularly, an improvement in reliability was observed at 500 Hz compared to the old-generation ASSR. This enhancement can be attributed to the utilization of narrow-band CE-chirp stimuli, which generate large amplitude responses, and the implementation of an improved detection paradigm involving multiple harmonics spectral and phase analysis. This pilot study only enrolled adults with normal hearing, and future investigations should include a larger sample size comprising both normal-hearing and hearing-impaired individuals, as well as the pediatric population. Full article

Fado is an urban Portuguese musical style rooted in popular culture. Previously found data suggests that Fado singers may have an increased risk of developing voice disorders. (1) Aim: To determine the risk factors for the development of voice disorders among Fado singers. (2) Methods: A cross-sectional study was conducted through the administration of a questionnaire containing questions related to voice disorders in singers. The relationship between personal and social data, musical background, performance demands and habits, vocal health and wellbeing, and strategies to overcome voice problems are reported. Beyond a comprehensive characterization, odds ratios (ORs) and their 95% confidence intervals (CIs) for the association with voice disorders were calculated through univariate and multivariate logistic regression analyses. (3) Results: The significant risk factors for voice disorders were as follows in decreasing order: nose-related disorders; decongestants or antihistamines; oral contraceptives or hormone replacement therapy; previous smoking habits; and vocal fatigue after performances. (4) Conclusion: These activities significantly increased the risk of developing voice disorders. The evidence from this study and the relative low prevalence of self-reported voice disorders suggest that these singers may develop a kind of protective combination of factors beyond the scope of this research. Full article

The subjective judgment on the annoyance produced by noise is always an important tool to complete an investigation, and to find the best way for its reduction. Therefore, acoustic analyses for environmental improvement frequently combine objective measures with subjective surveys to gain a comprehensive understanding of the problem. The technical specification, concerning the “Assessment of noise annoyance by means of social and socio-acoustic surveys”, ISO/TS 15666 (revised in 2021), has represented a basis for these activities since 2003, when it aimed to obtain information about noise annoyance “at home”. The more recent ISO/TS 12913-2 (2018), aimed at soundscape studies, investigations and applications, provides guidelines for conducting social and socio-acoustic surveys to assess noise annoyance and, in particular, it gives detailed indications on data collection and reporting. The indications provided by the technical specifications represent a common guideline for addressing the implementation of a subjective survey in the acoustic field, and to make investigations as comparable as possible. However, they do not cover all the fields of acoustic subjective investigations. Therefore, to make the collection of information from questionnaires more effective, it is useful to have a broader review of the issues that need to be addressed. To support this type of activity, this paper collects and presents relevant observations for the design of subjective survey questionnaires focused on outdoor acoustic quality. Drawing on previous research studies and experiences, it provides a series of observations on the main aspects to be considered in order to structure multipurpose acoustic evaluation questionnaires, including discussion of the general considerations for questionnaire structure, administration methods, population sample characterization, question formulation, and classification. It also deals with specific indications related to acoustic evaluations, such as the characteristics of annoying noise, boundary conditions, subjective feelings, and source features. Full article

Background: There is evidence that the cochlear volume may influence audiometric thresholds and CI electrodes’ impedance. The aim of the present study was to evaluate the impedance changes over time and correlate them to the residual volume of the cochlea. Methods: An MRI scan was performed via 3-D reconstruction before every surgery to obtain a residual volume for each ear. We performed repeated assessments of electrode impedance, both intra-operatively and post-implant, at the following intervals: 3 months, 6 months, and one year. The same type of perimodiolar array was implanted for each. Results: Thirty-four patients (10 (29.41%) male patients and 24 (70.59%) female patients) were evaluated. Patients received the implants between 2008 and 2017. The mean age of implantation was 13 ± 17.17 years, and the average of hearing thresholds improved after one year of the surgery. The mean cochlear volumes of the implanted ears were 68.16 ± 10.74 mm³ (right ear) and 56.54 ± 13.75 mm³ (left ear). We observed an increase in the basal electrodes’ impedance at the 3rd month. Yet, for the apical electrodes’ impedance, there was a decrease in averaged values. Conclusions: Post-operative impedance measurements were increased when compared to the intraoperatively measured basal values. Newly formed connective tissue is thought to be the cause of the higher impedance values. Full article

We report the case of a 51-year-old woman with multiple otologic and vestibular symptoms. She presented with two different types of tinnitus in her right ear, vertigo, and fluctuating aural symptoms in the left ear. She also complained of disequilibrium; chronic headache; hyperhidrosis; amenorrhea; insomnia; broadened hands and feet; and widened, thickened, and stubby fingers. The patient underwent careful collection of medical history, otomiscroscopy, pure tone audiometry, tympanometry, reflex threshold measurements, vestibular assessments, blood tests, magnetic resonance imaging (MRI), and cone beam computed tomography (CBTC) of the head. The audiogram showed: (1) a mild low-to-mid frequency conductive hearing loss, and a sharply sloping sensorineural hearing loss above 4000 Hz in the right ear; (2) a mild low-frequency sensorineural hearing loss in the left ear. MRI with 3D FLAIR sequences detected an acoustic neuroma (7.4 mm × 5.2 mm) in the middle-third of the right internal auditory canal, a pituitary macroadenoma (13 mm × 10 mm × 10 mm) and left saccular hydrops. The CBCT scan documented an outbreak of otosclerosis (3 mm) around the fissula ante fenestram in the right ear. Therefore, acoustic neuroma (right ear), growth hormone-secreting macroadenoma of the pituitary gland, Menière’s disease (left ear), and otosclerosis (right ear) were diagnosed/strongly suspected. A watch-and-wait strategy was adopted for acoustic neuroma and otosclerosis, while transsphenoidal surgery was successfully performed to remove the pituitary macroadenoma. This case report confirms that multiple otologic disorders can occur simultaneously in the same patient, requiring prompt audiological and imaging evaluations. Full article

Background: The goal of this study is the identification of potential marker molecules for characterizing different basal cell carcinoma entities, to help improve clinical decisions for surgical resection therapy. Methods: Three different entities, sclerodermiform, solid and superficial basal cell carcinomas, were subjected to immunohistochemical microscopy and histomorphometric analyses for human α- (DEFA1/3; DEFA4) and β-defensins (hBD1/2/3) and special S100 proteins (S100A4/7/8/9). Thirty specimens of the three entities were evaluated. Analyses were performed by comparing tissue and cellular localization and staining intensities of tumorous with non-tumorous areas. Staining intensities were semiquantitatively examined by using an RGB-based model. Results: Human defensins are present in all three entities of basal cell carcinomas. They all show cytoplasmic immunostaining in cells of the epithelium, stroma and tumor. Notably, human β-defensin3 is accumulated in the cell nuclei of sclerodermiform and superficial basal cell carcinomas. S100A4 and A7 are undetectable in tumor regions. However, S100A4 occurs in cancer-associated stroma cells with nuclear staining in superficial basal cell carcinomas. Conclusion: Two candidates, namely hBD3 and S100A4, might be used as potential clinical tools for evaluating successful surgical resection therapy to avoid aesthetic and functional facial deformation. Full article

This study examines the relationship between the early identification of hearing loss and language outcomes for deaf/hard of hearing (D/HH) children, with bilateral or unilateral hearing loss and with or without additional disabilities. It was hypothesized that hearing loss identified by 3 months of age would be associated with better language outcomes. Using a prospective, longitudinal design, 86 families completed developmental instruments at two time points: at an average age of 14.8 months and an average age of 32.1 months. Multiple regression examined how hearing loss identified by 3 months of age contributed to later language outcomes while controlling for developmental level at the first time point. Hearing loss identified by 3 months of age was positively associated with better language outcomes for D/HH children at 32 months of age; however, D/HH children still exhibited language delays, compared to normative scores for same-aged hearing peers for reported measures. Language outcomes of children with unilateral hearing loss were not better than those of children with mild-to-moderate bilateral hearing loss. Children with additional disabilities and more severe bilateral hearing loss had lower language scores than those without. Full article

The application of machine learning (ML) techniques to otolaryngology remains a topic of interest and prevalence in the literature, though no previous articles have summarized the current state of ML application to management and the diagnosis of lateral skull base (LSB) tumors. Subsequently, we present a systematic overview of previous applications of ML techniques to the management of LSB tumors. Independent searches were conducted on PubMed and Web of Science between August 2020 and February 2021 to identify the literature pertaining to the use of ML techniques in LSB tumor surgery written in the English language. All articles were assessed in regard to their application task, ML methodology, and their outcomes. A total of 32 articles were examined. The number of articles involving applications of ML techniques to LSB tumor surgeries has significantly increased since the first article relevant to this field was published in 1994. The most commonly employed ML category was tree-based algorithms. Most articles were included in the category of surgical management (13; 40.6%), followed by those in disease classification (8; 25%). Overall, the application of ML techniques to the management of LSB tumor has evolved rapidly over the past two decades, and the anticipated growth in the future could significantly augment the surgical outcomes and management of LSB tumors. Full article