Threshold Audiometry Research Articles

Ototoxicity associated with hematopoietic stem cell transplantation; what are the risk factors?

Background Hematopoietic stem cell transplantation (HSCT) is a critical treatment for various hematologic malignancies but can lead to complications, including ototoxicity. Aim/objectives This study aims to explore the relationship between patient-specific factors and ototoxicity in adult HSCT patients. Material and Methods We conducted a retrospective analysis of 129 adult patients who underwent HSCT between 2003 and 2020. Age, gender, transplant indications, conditioning regimens, and pre- and post-transplant audiometry thresholds data were collected from patient files. A hearing loss of 10 decibels or more at two consecutive frequencies or a hearing loss of 20 decibels or more at a single frequency was considered as significant hearing loss (SHL). Statistical analyses were performed to describe factors associated with SHL. Results SHL occurred in 16.3% of patients. Older age was significantly associated with an increased risk of SHL (p = .035). Poorer pretransplant hearing thresholds at 4000 Hz and 6000 Hz were also significant predictors of SHL (p = .039 and p = .014, respectively). There was no significant relationship between the donor type of HSCT (autologous vs. allogeneic) and ototoxicity (p = .45), and between conditioning regimens and ototoxicity (p = .860). Conclusions Age and pre-existing hearing levels are significant predictors of ototoxicity post-HSCT. Careful management and monitoring are essential to prevent and address hearing loss in HSCT patients to improve hearing-related quality of life.

Beyond the cochlea: exploring the multifaceted nature of hearing loss in primary mitochondrial diseases

Abstract Primary mitochondrial diseases, with diverse systemic manifestations, often present with auditory impairments due to mitochondrial dysfunction. This study provides an in-depth exploration of auditory deficits in primary mitochondrial diseases, highlighting the impact of various pathogenic variants on both cochlea and neural/central auditory functions. An observational study involving 72 adults with primary mitochondrial diseases was conducted. Participants underwent extensive audiological evaluations including Pure-Tone Audiometry, Tympanometry, Acoustic Reflex Thresholds, Quick Speech-in-Noise Test, Listening in Spatialized Noise-Sentences test, Auditory-evoked Brainstem Responses, and Distortion Product Otoacoustic Emissions. Multivariate Analysis of Covariance and logistic regression analyses assessed the influence of various pathogenic DNA variants, accounting for age, cognitive status via the Montreal Cognitive Assessment, and disease severity through the Newcastle Mitochondrial Disease Adult Scale. Participants with the pathogenic m.3243A>G/T variants (m.3243A>G n=40; m.3243A>T n=1) exhibited significant elevations in Pure-Tone Audiometry thresholds, especially at high frequencies, suggesting cochlea involvement. Notably, the Listening in Spatialized Noise-Sentences test showed significant spatial processing deficits in the m.3243A>G/T group, possibly indicating a unique mutation-specific impact on central auditory processing. Auditory-evoked Brainstem Responses results highlighted a higher likelihood of auditory brainstem response abnormalities in this group, further substantiating neural/central auditory pathway involvement. This study emphasises the heterogeneous nature of hearing impairment in primary mitochondrial diseases, with a genotype-phenotype correlation, particularly in the m.3243A>G/T group. These insights advocate for personalised, genotype-specific auditory assessments and targeted management strategies. Conventional hearing aids and cochlear implants are ineffective for those with central auditory dysfunctions related to mitochondrial mutations. There is an urgent need for innovative rehabilitation strategies catering for both cochlear and neural/central auditory pathways.

Diagnosis of auditory disorders in dental patients with altered occlusal relationships (Part 1)

Relevance. Recently, dental patients have been increasingly presenting with not only aesthetic, functional, and painrelated concerns, but also various otological symptoms, such as ear congestion, discomfort, and perceived hearing loss. These symptoms may be linked to underlying dental conditions, especially those involving a reduction in interalveolar distance (IAD), and must be carefully differentiated from ENT disorders and true hearing impairment.Objective: To evaluate the auditory function in dental patients with partial tooth loss and complaints of pathological otological symptoms.Materials and methods. The study included 357 dental patients aged 43 to 72 years, comprising 287 women (80.4%) and 70 men (19.6%) with partial tooth loss. The examination involved a functional-physiological method for determining the central relationship of the jaws using the Apparatus for Determining Central Occlusion (AOTSO) and tonal threshold audiometry.Results. A reduction in interalveolar height of varying degrees was confirmed in 325 (91%) patients with dental arch defects, while no decrease was observed in 23 (9%) patients. All patients with otological complaints exhibited a reduction in lower facial height. Of the 87 patients with otological complaints, 37 (42.5%) were diagnosed with significant hearing loss based on tonal threshold audiometry.Conclusion. The examination of this group of dental patients with dental arch defects, utilizing modern diagnostic methods, confirmed a reduction in interalveolar distance. Additionally, otological complaints were identified, and pathological auditory conditions, such as various forms of hearing loss, were verified through tonal threshold audiometry.

The biphasic impact of apolipoprotein E ε4 allele on age-related hearing loss

Both the ε4 variant of the apolipoprotein E (APOE) gene and hearing loss are well-known risk factors for Alzheimer's disease. However, previous studies have produced inconsistent findings regarding the association between APOE genotypes and hearing levels, necessitating further investigation. The aim of this study was to investigate the relationship between APOE genotypes and hearing levels. This retrospective study analyzed clinical data from a clinical data warehouse of seven affiliated Catholic Medical Center hospitals. The study included 1,162 participants with records of APOE genotypes, audiometric tests, and cognitive function tests. In Generalized linear mixed model analysis, ε4 carriers exhibited lower pure tone audiometry thresholds with an estimate of -0.353 (SE = 0.126, p = 0.005). However, the interaction term for age and APOE ε4 had a coefficient of 0.577 (SE = 0.214 p = 0.006), suggesting that the APOE ε4 gene may accelerate hearing deterioration with age. Subgroup analysis based on an age cut-off of 75 revealed that ε4 carriers had better hearing at younger ages, but showed no significant difference at older ages. These results indicate that the ε4 allele may have a biphasic effect on hearing levels depending on age.

Estimation of Hearing Thresholds with Auditory Steady-State Responses to Narrow-Band Chirps in Children

Objective: The aim of this study was to evaluate the utility of auditory steady-state responses (ASSRs) to narrow-band chirps (NB-chirps) for estimating hearing levels in children. Design: Thresholds from the NB-chirp ASSR were evaluated in 30 sedated children with normal hearing or hearing loss. The correlation between the NB-chirp ASSR and pure-tone audiometry (PTA) thresholds was analyzed, and the difference score (DS) between these thresholds was calculated. Data from the NB-chirp ASSR were compared to retrospective data from conventional ASSR to exponentially amplitude-modulated tones in 25 sedated children. Results: Positive correlations between the NB-chirp ASSR and PTA thresholds were found at 500, 1,000, 2,000, and 4,000 Hz. Multiple comparisons of the DSs for the NB-chirp ASSR and PTA revealed significant differences at 500–2,000 Hz and 4,000 Hz, as well as 1,000–2,000 Hz, and 4,000 Hz. Comparisons of the DSs demonstrated that the DS of the NB-chirp ASSR was significantly smaller than that of the conventional ASSR at 2,000 Hz. Furthermore, the testing times for the NB-chirp ASSR were significantly shorter than those for the conventional ASSR. Conclusion: The NB-chirp ASSR closely reflected the PTA thresholds, and the testing time was shorter than that of the conventional ASSR. Thus, this study demonstrated that the NB-chirp ASSR is useful for hearing threshold estimation in children.

Measurement of thresholds using Chirp-ABR in children with auditory neuropathy spectrum disorder and sensorineural hearing loss

AimThis study explored the value of Chirp-auditory brainstem response (ABR) thresholds in assessing the hearing threshold of children diagnosed with auditory neuropathy spectrum disorder (ANSD). MethodsA total of 20 children with ANSD (40 ears, aged 1.5–7.0 years, median age 4.5 years) and 31 children with sensorineural hearing loss (SNHL) (52 ears, aged 0.9–8.0 years, median age 3.7 years) were included. Besides, 25 normal children (50 ears, aged 0.8–7.5 years, median age 4.6 years) were used as controls. Chirp-ABR and behavioral audiometry were performed simultaneously among three groups of children, allowing for a comparison of the thresholds obtained through both methods. ResultsIn ANSD children, the correlation (r-values) between the thresholds obtained from Chirp-ABR and behavioral audiometry at 500–4000 Hz were 0.84, 0.67, 0.59, and 0.60, respectively. The average threshold differences between two methods ranged from 9.7 to 13.3 dB at 500–4000 Hz. Notably, 20 % ears (8/40) exhibited considerable discrepancies (>30 dB) in thresholds at certain frequencies. For SNHL children, the r-values between two methods were 0.84, 0.89, 0.92, and 0.93, respectively. The average threshold differences between two methods were 5.7–8.2 dB at 500–4000 Hz. Similarly, in normal children, the average threshold differences between two methods ranged from 6.1 dB to 7.7 dB, the r-values were 0.81, 0.78, 0.80, and 0.80 at 500–4000 Hz, respectively. ConclusionChirp-ABR threshold is not suitable to predict the behavioral audiometry threshold in ANSD children. When there is a significant discrepancy (>30 dB) between Chirp-ABR thresholds and behavioral audiometry thresholds in hearing loss, ANSD should be highly suspected.

Visual selective attention in individuals with age-related hearing loss

Evidence from epidemiological studies suggests that hearing loss is associated with an accelerated decline in cognitive function, but the underlying pathophysiological mechanism remains poorly understood. Studies using auditory tasks have suggested that degraded auditory input increases the cognitive load for auditory perceptual processing and thereby reduces the resources available for other cognitive tasks. Attention-related networks are among the systems overrecruited to support degraded auditory perception, but it is unclear how they function when no excessive recruitment of cognitive resources for auditory processing is needed. Here, we implemented an EEG study using a nonauditory visual attentional selection task in 30 individuals with age-related hearing loss (ARHLs, 60–73 years) and compared them with aged (N = 30, 60–70 years) and young (N = 35, 22–29 years) normal-hearing controls. Compared with their normal-hearing peers, ARHLs demonstrated a significant amplitude reduction for the posterior contralateral N2 component, which is a well-validated index of the allocation of selective visual attention, despite the comparable behavioral performance. Furthermore, the amplitudes were observed to correlate significantly with hearing acuities (pure tone audiometry thresholds) and higher-order hearing abilities (speech-in-noise thresholds) in aged individuals. The target-elicited alpha lateralization, another mechanism of visuospatial attention, demonstrated in control groups was not observed in ARHLs. Although behavioral performance is comparable, the significant decrease in N2pc amplitude in ARHLs provides neurophysiologic evidence that may suggest a visual attentional deficit in ARHLs even without extra-recruitment of cognitive resources by auditory processing. It supports the hypothesis that constant degraded auditory input in ARHLs has an adverse impact on the function of cognitive control systems, which is a possible mechanism mediating the relationship between hearing loss and cognitive decline.

OUR EXPERIENCE IN SURGICAL TREATMENT OF MUCOSAL HYPERTROPHY OF TORI TUBARII

Puprose. Evaluation of the effectiveness of surgical treatment of mucous hypertrophy of tori tubarii by submucosal tissue destruction using Ho:YAG laser radiation. Materials and methods. The study involved 48 adult patients with symptoms of Eustachian tube dysfunction. The patency of the auditory tube was assessed by standard tests, the ETDQ 7 questionnaire, tympanometry, pure tone threshold audiometry, and endoscopic video recording of the results were performed. Surgical intervention was carried out by forming under the influence of pulsed radiation from a Ho:YAG laser with a frequency of 10 Hz and a pulse energy of 2 J, 4–6 tunnels with a depth of 5–7 mm in the hypertrophied mucous membrane of the torui tubarii along the posteromedial surface and 2 tunnels up to 3 mm up and down from the pharyngeal mouth of the auditory tube. The control periods after surgery were 1, 3 and 6 months. Results. A significant improvement in the results of the auditory tube according to ETDQ-7 was noted by 3 months after surgery in 71% of patients and in 84% by 6 months. By 6 months after surgery, type C tympanometry and clinically significant conductive hearing loss persisted in 8 and 5% of patients, respectively. The subjective feeling of fully performing the Valsalva test improved in 58% after 3 months and in 82% by the 6th month, and the Toynbee test in 42% and 64% in the 3rd and 6th months, respectively. In 16% of patients, despite a visual decrease in mucosal hypertrophy during endoscopic examination, signs of dysfunction remained according to ETDQ 7. There were no complications after surgical treatment during 6 months of observation. Conclusion. The use of endoscopic diagnosis of the nasopharynx in patients with signs of dysfunction of the auditory tubes in combination with functional tests and the ETDQ-7 questionnaire allows to determine the indications for the surgical treatment of hypertrophy of the mucous membrane of the tori tubarii using the Ho:YAG laser, the effectiveness of which in our study was 84%.

Effect of mecobalamin combined with holographic copper acupuncture and scraping technique on tinnitus symptoms in patients with sudden sensorineural hearing loss

Purpose: To examine the effect of methylcobalamin combined with holistic copper acupuncture and scraping technique in ameliorating tinnitus symptoms in patients with sudden sensorineural hearing loss. Methods: 80 patients with sudden sensorineural hearing loss from Department of Otolaryngology, The Hefei First People’s Hospital, Hefei, China were selected as subjects for this study. They were divided into control and study groups, respectively, with 40 patients in each group, based on the different treatment interventions received by the patients. Conventional interventions included the use of Ginkgo biloba extract, Tianmasu injection, dexamethasone pharmacological treatments. The control group received methylphenidate tablets in addition to the conventional treatment, while the study group received further intervention comprising the combination of holistic copper acupuncture and scraping technique on the ear, in addition to the treatment received by the control group. The evaluated outcomes included efficacy, auditory threshold levels, and Pittsburgh Sleep Quality Index (PSQI) score. Results: The overall efficacy of the treatment in the control group was 77.5 %, whereas, it was significantly higher in the study group at 97.5 % (p < 0.05). Prior to treatment, there were no significant differences in pure tone audiometry thresholds between the two groups (p > 0.05). However, post-treatment, the study group exhibited significantly lower pure tone audiometry thresholds compared to the control group (p < 0.05). Before PSQI scores were similar for the two groups (p > 0.05). However, after treatment, the PSQI scores in the study group were significantly lower than those in the control group (p < 0.05). Conclusion: The combined application of methylcobalamin, holistic copper acupuncture, and scraping technique is effective for the treatment of patients with sudden sensorineural hearing loss. This integrated treatment approach significantly alleviates tinnitus and improves sleep-related symptoms in patients with sudden sensorineural hearing loss. Furthermore, it promotes the recovery of pure tone audiometry thresholds and other related parameters.

Hearing health survey of the population in Bangkok

In this cross-sectional random survey among Thai adults living in Bangkok, we aimed to identify the prevalence of hearing problems and examine their relationship with individual factors. We administered a self-report questionnaire and performed pure-tone air conduction threshold audiometry. A total of 2463 participants (1728 female individuals) aged 15–96 years were included. The hearing loss prevalence was 53.02% and increased with age. The prevalence of a moderate or greater degree of hearing impairment was 2.8%. Participants aged 65 years and over had 8.56 and 6.79 times greater hearing loss and hearing impairment than younger participants, respectively. Male participants were twice as likely to have hearing loss and hearing impairment as female individuals. Participants with higher education levels showed less likelihood of having hearing loss and hearing impairment than those with no or a primary school education. Participants who ever worked under conditions with loud noise for > 8 h per day had 1.56 times greater hearing loss than those without such exposure. An inconsistent correlation was found between hearing loss, hearing impairment and noncommunicable diseases (diabetes, hypertension, and obesity). Although most participants had mild hearing loss, appropriate care and monitoring are necessary to prevent further loss in such individuals. The questionnaire-based survey found only people with hearing problems that affect daily communication.

Standard and Extend High-Frequency Audiometry in Sudden Sensorineural Hearing Loss: Impacts on Tinnitus and Mental Health.

To analyze the results of auditory assessment in standard (SA) and extended high-frequency (EHF) audiometry, associating the findings with sudden tinnitus and mental health of patients with unilateral sudden sensorineural hearing loss (SSNHL). Prospective, cohort study. Outpatient otology clinic in a tertiary care hospital. Patients experiencing unilateral SSNHL were evaluated with pure-tone audiometry performed at frequencies of 250 to16,000 Hz, tinnitus pitch and loudness matching tests, Tinnitus Handicap Inventory (THI), Analog and Visual Scale (AVS) for bothersome tinnitus, and the Hospital Anxiety and Depression Scale (HADS). Eighteen patients with unilateral SSNHL were assessed. After starting treatment, there was a significant improvement in the SA (71.1 dB to 50 dB; p < 0.001*) and EHF audiometry (64.5 dB to 54.4 dB; p < 0.001*) thresholds at 15 days, and this persisted at 30 days of follow-up. Significant improvements were seen for tinnitus in loudness, VAS, and THI and for mental health in the realms of anxiety and depression by HADS. Despite improvements in SA, persistent EHF hearing loss was accompanied by persistent tinnitus, but it was of diminished loudness. Despite improvement in pure-tone thresholds by SA, a subset of unilateral SSNHL patients did not experience hearing recovery in EHF thresholds and reported persistent tinnitus. We postulate that their diminished anxiety and better mental health may be related to both hearing improvement in standard audiometry and reduction in tinnitus loudness. This pilot prospective study investigates the utility of performing EHF audiometry to better understand outcomes in patients with SSNHL.

Self-reporting of hearing loss and tinnitus in the diagnosis of ototoxicity by meglumine antimoniate in patients treated for American tegumentary Leishmaniasis.

American Tegumentary Leishmaniasis (ATL) treatment is based on pentavalent antimonials (Sb5+), but these drugs have been associated to several adverse effects. Hearing loss and tinnitus during treatment with meglumine antimoniate (MA) have already been reported. This study aimed to describe the usefulness of self-reporting of hearing loss and tinnitus in diagnosing MA-induced ototoxicity. A prospective longitudinal study was conducted with 102 patients with parasitological diagnosis of ATL, treated with different MA schemes. The presence of clinical auditory toxicity was defined as the emergence or worsening of self-reporting hearing loss and/or tinnitus during monitoring. Measures of sensitivity, specificity, and the positive and negative predictive value of the patient's self-reporting of hearing loss and tinnitus in relation to the result of the audiometric test (considered the gold standard) were calculated. The age of the evaluated patients ranged from 15 to 81 years, with a median of 41 years, and most were male (73.5%). Seventy-five patients (73.5%) had cutaneous leishmaniasis and 27 (26.5%) mucosal leishmaniasis. Eighty-six patients (84.3%) received intramuscular (IM) treatment and 16 (15.7%) were treated with intralesional MA. During treatment, 18 (17,6%) had tinnitus and 7 (6,9%) had complaint of hearing loss. 53 (52%) patients had cochlear toxicity confirmed by tone threshold audiometry and high frequency audiometry, from which 60% received a dose of 20 mg Sb5+/kg/day (p = 0.015) and 96.2% were treated with IM MA (p = 0.001). Tinnitus has greater specificity and positive predictive value than hearing loss, with a low number of false positives, but with a high false negative value. Although the large number of false negatives suggests that self-report of hearing loss or tinnitus cannot be considered a good screening test for referring the patient to an audiometry, the low number of false positives suggests the need to value the patient's complaint for referral. Otherwise, this study reinforces the importance of audiological monitoring during treatment with MA, especially in those patients with self-reporting of hearing loss or tinnitus when treated with 20 mg Sb5+/kg/day via IM.

Psychophysical and Electrophysiological Parameters of the Auditory Analyzer as Indicators of the Effectiveness of Cochlear Implantation in Children with Bilateral Deafness

Background. The results of rehabilitation of patients with deafness after cochlear implantation (CI) depends on many factors related on both the individual characteristics of the patient and the technical parameters of auditory nerve stimulation — fitting of the CI system processor. Article presents the results of a study of the relationship between psychophysical (subjective) auditory sensations and electrophysiological (objective) responses of the structures of the auditory analyzer to electrical stimulation. Aims — to evaluate the relationship between the electrophysiological responses of the auditory analyzer and the psychophysical parameters of the thresholds of sound perception and speech intelligibility in patients with deafness after CI. Methods. The study involved 88 patients with IV bilateral sensorineural hearing loss, borderline with deafness, aged 6 to 17 years 11 months, among whom 54.5% (n = 48) of girls and 45.5% (n = 40) of boys. Of the patients included in the follow-up, 40% (n = 35) underwent unilateral CI; 60% (n = 53) of children underwent bilateral sequential CI. In our study, slightly more than half of the patients (51.1%) had experience using the CI system for more than 5 years. At the first stage of the study, we studied the relationship between sound perception thresholds (tonal audiometry in a free field) and registered ASSR (auditory steady state response) thresholds in patients with deafness receiving rehabilitation after CI surgery. The relationship between behavioral and electrophysiological (objective) thresholds was assessed by comparing the results of tonal threshold audiometry and the responses received during ASSR registration to acoustic stimulation in a free field. At the second stage, patients with indications for processor fitting (decreased speech intelligibility, uncomfortable sensations) underwent speech audiometry, evoked compound action potentials (ECAP) registration, according to the results of which the parameters of the CI processor were changed. Results. Research data showed the effectiveness of the approach of including the ECAP registration test, followed by the creation of a fitting card and correction of the level of auditory nerve stimulation under the control of speech audiometry in the protocol of fitting the CI processor. Conclusions. The results of the conducted studies allow us to recommend the use of the developed method of objective recording of sound perception thresholds in patients with CI as one of the indicators of the effectiveness of CI in the early period of auditory-speech rehabilitation in patients with undeveloped speech skills and when it is impossible to conduct objective tests accompanied by supra-threshold stimulation of the auditory nerve.

A Multicenter Validity Study of Four Smartphone Hearing Test Apps in Optimized and Home Environments.

Pure tone audiometry (PTA) is the gold standard for hearing assessment. However, it requires access to specialized equipment. Smartphone audiometry applications (apps) have been developed to perform automated threshold audiometry and could allow patients to perform self-administered screening or monitoring. This study aimed to assess the validity and feasibility of patients using apps to self-assess hearing thresholds at home, with comparison to PTA. A multi-center, prospective randomized study was conducted amongst patients undergoing PTA in clinics. Participants were randomly allocated to one of four publicly-available apps designed to measure pure tone thresholds. Participants used an app once in optimal sound-treated conditions and a further three times at home. Ear-specific frequency-specific thresholds and pure tone average were compared using Pearson correlation coefficient. The percentage of app hearing tests with results within ±10 dB of PTA was calculated. Patient acceptability was assessed via an online survey. One hundred thirty-nine participants submitted data. The results of two at-home automated smartphone apps correlated strongly/very strongly with PTA average and their frequency-specific median was within ±10 dB accuracy. Smartphone audiometry performed in sound-treated and home conditions were very strongly correlated. The apps were rated as easy/very easy to use by 90% of participants and 90% would be happy/very happy to use an app to monitor their hearing. Judicious use of self-performed smartphone audiometry was both valid and feasible for two of four apps. It could provide frequency-specific threshold estimates at home, potentially allowing assessments of patients remotely or monitoring of fluctuating hearing loss. 2 Laryngoscope, 134:2864-2870, 2024.

Umbrales estimados (esdB HL) para potenciales evocados auditivos con estímulo Narrow Band CE-Chirp®

ABSTRACT Purpose: to correlate pure-tone audiometry thresholds with electrophysiological thresholds, using NB CE-Chirp® stimuli in listeners aged 18 to 30 years from Santiago, Chile, and determine the estimated threshold at 500, 1000, 2000, and 4000 Hz. This can contribute to the battery of objective and subjective tests applicable to children, aiding the timely detection and implementation before 3 months old, as recommended by the Joint Committee on Infant Hearing. Methods: a quantitative, nonexperimental study with a sample of 30 hearing subjects, aged 18 to 30 years. The Pearson´s correlation test was applied, with a significance p-value of 0.01. Results: higher frequencies (2000 and 4000 Hz) have lower correction factors than medium and lower frequencies (1000 and 500 Hz), which tend to be higher as the frequency is lower. Conclusions: the study obtained the following estimated thresholds: 15 dB at 500 Hz, 10 dB at 1000 and 2000 Hz, and 5 dB at 4000 Hz.

Establishment of the Norwegian hearing register for children.

The Norwegian Directorate of Health approved the Norwegian Hearing Register for Children in 2022. The main objective of the register is to improve the quality of treatment for children with permanent hearing loss, by measures, follow-ups and monitoring the quality and results of the health care system. Inclusion criteria are children who do not pass universal newborn hearing screening and/or children with permanent hearing loss <18 years of age. Hearing loss is defined as pure-tone audiometry threshold of (PTA4) > 20 dB in at least one ear. Data are registered at the Ear, Nose and Throat departments at inclusion and at follow-ups at the age of 3, 6, 10, and 15 years. The register collects information about the child within a holistic perspective. The key elements of the register are (a) data concerning newborn hearing screening; (b) data concerning hearing, medical information, hearing amplification and intervention (c) patient reported outcome measures registered by caregivers using three questionnaires; Pediatric Quality of Life Inventory, Strengths and Difficulties Questionnaire and Parents' Evaluation of Aural/Oral Performance of Children. The register has established four quality indicators regarding newborn hearing screening and early intervention (a) the rate of false positive neonatal screens; (b) testing for congenital cytomegalovirus within 3 weeks of age for children who do not pass newborn hearing screening; (c) audiological evaluation to confirm the hearing status no later than 3 months of age and (d) initiated intervention within 3 months after confirmation of hearing status. The register will include the total population of hearing impaired children over long time periods. Thus, the register enables each hospital to monitor their quality indicator scores continuously and compare them with national levels in real time. This facilitates and accelerates identification of improvement areas in the hospitals and will be an important contributor for quality improvement in NHS, diagnostics and hearing intervention for children in Norway. In addition, data from the register will be a unique source for research, and study designs with a long follow-up time can be applied.

Assessment of the risk of hearing loss in children with cystic fibrosis

Cystic fibrosis (CF) is a severe hereditary disease with a multisystem lesion. Manifestations of CF include severe infectious purulent lesions of all parts of the respiratory tract, including purulent rhinosinusitis with nasal polyps. The involvement of the sinonasal region and the need for systemic use of ototoxic drugs (primarily aminoglycosides to treat resistant bacterial infection) potentially create a risk of both conductive and sensorineural hearing loss (SNHL). The available data on the epidemiology of hearing disorders in CF is contradictory. Currently, genetic determinants of the development of aminoglycoside SNHL have been identified. For 136 CF patients (75 girls, 61 boys) aged 3 to 17 (9.4±3.9) years were performed audiological examination: tympanometry, transient-evoked otoacoustic emission and the pure tone threshold audiometry (standard frequency range) (n=126). History of systemic therapy with aminoglycosides was evaluated for each patient. Sequencing of c.35delG mutations in the GJB2 gene (nuclear DNA) and A1555G in the 12S rRNA gene (mitochondrial DNA) was performed in 215 patients with cystic fibrosis (the group partially overlaps with the audiological group), and as a control - 106 children with bronchial asthma and 103 healthy children, their age ranged from 3 to 17 (8.8±3.8) years. Audiological examination of CF children reveled a prevalence of conductive hearing loss comparable to the general population (2.4%). The frequency of SNHL was 1.6%, wich exceeds that of non-CF children. A genetic study revealed one case of heterozygous carriage of the c.35delG mutation in the GJB2 gene in a patient with bronchial asthma. In the group of patients with CF (n=215), mutations in the connexin 26 gene were not detected. No A1555G mutation was detected either in the group of patients with CF or in the control groups. Children with CF are at risk for the development of sensorineural, but not conductive hearing loss. Routine total screening for A1555G and c.35delG mutations probably seems not to be recommended.

Comparative Analysis of Methods of Audiological Screening of the Adult Population: Global and Kazakhstani Experience

Hearing impairment is currently recognized as the most prevalent sensory impairment and one of the leading causes of disability worldwide. It is estimated that one in five people on Earth has at least minimal hearing loss, and more than 5% of the population has disabling hearing loss. There are many methods for screening detection of hearing impairment, and the most common ones are mobile applications, recognition of whispered speech, a portable audiometer, questionnaires, the speech intelligibility test in noise, and others. Nevertheless, the “gold standard” for hearing testing has been and remains the method of pure-tone threshold audiometry. The purpose of this review is to study the currently available literature, analyze ongoing research in the field of audiological screening of adults, study the cost-effectiveness of ongoing screening activities, study the situation in Kazakhstan, and the possibility of using these screening methods in Kazakhstan. We selected 25 full-text articles that met the following criteria: the 2018-2023 time interval, adult population, screening methods, effectiveness, and article language (English, Russian, Kazakh). Conclusions. Despite a sufficient number of hearing screening methods, their sufficient reliability, sensitivity, and specificity, and their obvious cost-effectiveness in comparison with the costs of rehabilitation of hearing impairments, none of the hearing assessment methods are included in the National Screening Program for examining adult population in any country in the world. In Kazakhstan, a screening test of hearing function using a one-question survey, the HHIA/HHIE questionnaire, a study of whispered speech at the primary health care level, and also the use of a mobile version of a hearing test as a hearing self-diagnosis are applicabl

Plasma Levels of Interferon Gamma associated with Hearing Loss and Hearing Loss Sensation through the Handicap Questionnaire Inventory for the Elderly Screening Version.

The aim of this study was to verify the association between the auditory handicap found in the Hearing Handicap Inventory for the Elderly-Screening Version (HHIE-S) questionnaire and hearing loss and the plasma levels of inflammatory biomarkers. Cross-sectional study with 76 participants, 67 (88%) females and 9 (12%) males, with a mean age of 70 years. Tonal threshold audiometry and self-assessment with HHIE-S questionnaire were performed to measure the plasma levels of interleukin-2 (IL-2), IL-4, IL-6, and IL-10; tumor necrosis factor alpha; and interferon gamma (IFN-γ) flow cytometry method. For all data analyzed, the significance level adopted was P < 0.05 and 95% confidence interval. An inverse correlation was observed between the increase in plasma levels of IFN-γ and normal auditory handicap (P = 0.015; rs = -0.280). The severe handicap group showed an increase in the averages I (P = 0.005; rs = 0.350) and II (P = 0.016; rs = 0.368) in the right ear and the light/moderate handicap group increased the means I (P = 0.027; rs = 0.350) and II (P = 0.046; rs = 0.310) of the left ear. A statistically significant association was found between the speech recognition threshold (SRT) test results of the right ear and the severe handicap group (P = 0.002; rs = 0.271). There was an association between the increase in plasma levels of IFN-γ and normal auditory handicap. Additionally, statistically significant associations were observed between the mild/moderate and severe handicap groups with the increase in hearing means and an increase in SRT associated with the severe handicap group.

Comparisons of air-conduction hearing thresholds between manual and automated methods in a commercial audiometer.

To investigate the correlation of air-conduction thresholds between automated audiometry in a non-isolated environment and manual audiometry in participants with normal hearing and different degrees of hearing loss. Eighty-three participants aged 11-88 years old underwent automated pure-tone audiometry in a non-acoustically isolated environment, and the results were compared with those of manual pure-tone audiometry performed in a standard acoustically isolated booth, with the order of testing randomised. Six frequencies of 250, 500, 1,000, 2000, 4,000 and 8,000 Hz were tested. All 166 ears were completed and 996 valid hearing threshold data were obtained, with 28 data exceeding the 95% confidence interval in the Bland-Altman plot, accounting for 2.81% of all data. The means and standard deviations of the differences for the six frequencies from 250 to 8,000 Hz were, respectively, 0.63 ± 5.31, 0.69 ± 4.50, 0.45 ± 4.99, 0.3 ± 6.2, -0.15 ± 4.8, and 0.21 ± 4.97 dB. The correlation coefficients of the two test results for normal hearing, mild, moderate, severe and above hearing loss groups were 0.95, 0.92, 0.97, and 0.96, respectively. The correlation coefficient of the automated and manual audiometry thresholds for the age groups under 40 years, 40-60 years, and 60 years above, were 0.98, 0.97 and 0.97, respectively, with all being statistically significant (p < 0.01). The response time of the three age groups were 791 ± 181 ms, 900 ± 190 ms and 1,063 ± 332 ms, respectively, and there was a significant difference between the groups under 40 years and over 60 years. There was good consistency between automated pure-tone audiometry in a non-acoustically isolated environment and manual pure-tone audiometry in participants with different hearing levels and different age groups.