Search Results (47)

Background: This study examined the relationship between hearing device price and sound quality. Method: A novel consumer-centric metric of sound quality (“SoundScore”) was used to assess hearing devices’ audio performance. Each hearing device is tested with two fittings. The “Initial Fit” is designed to approximate the most likely fitting for an individual with a mild-to-moderate sloping sensorineural hearing loss. The “Tuned Fit” includes adjusting parameters optimized to hit prescriptive fitting targets (NAL NL2) on an acoustic manikin. Each fitting is evaluated across five dimensions. Both fittings are combined using a weighted average to create a single number from 0 to 5 representative of a device’s overall audio performance. Seventy-one hearing devices were tested. Results: A strong positive correlation was found between hearing device price and SoundScore. The average SoundScore increased dramatically as the price approached USD 1000, with marginal improvements beyond this point. SoundScore was consistently poor for devices under USD 500, highly variable between USD 500–1000, and consistently good over USD 1000. Conclusions: There is a strong but nonlinear relationship between hearing device price and sound quality. This information can aid consumers in making informed decisions while also assisting hearing healthcare professionals in providing comprehensive guidance to their patients. Full article

Background/Objectives: Hearing loss is one of the most common sensorineural impairments, and approximately 60% of early-onset cases are due to genetic variations. The otogelin-like protein, encoded by the OTOGL gene, is a component of the acellular membranes of the inner ear, such as the tectorial membrane, and is thought to play an important role in cochlear amplification. OTOGL gene variants are a rare cause of hearing loss such as DFNB84B, a mild-to-moderate sensorineural hearing loss presenting in early childhood with autosomal recessive inheritance. In this study, we aim to enhance our comprehension of the phenotypes of hearing loss caused by OTOGL variants. Methods: A total of 7056 Japanese patients with hearing loss were recruited, and based on massively parallel DNA sequencing on 158 target genes, we selected patients with biallelic OTOGL variants. Results: Ten affected individuals with OTOGL gene variants were detected, the largest group of patients yet to be reported, and eight of the eleven variants were novel. Our results showed that variations in this gene led to mild-to-moderate non-progressive hearing loss, and the accompanying symptoms, mainly vestibular symptoms, were speculated to present in adulthood. Conclusions: Determination of the phenotypes of genes causative of hearing loss is expected to greatly benefit patients with hearing loss as it can assist in predicting outcomes and lead to appropriate intervention, which, in OTOGL-associated hearing loss cases, is based around the fact that the patients need not be concerned with deterioration in hearing, but require careful follow-up for vestibular symptoms. Full article

Background: When speaking while wearing a face mask, sound transmission is attenuated, and visual cues are lost due to the covered facial movements of the speaker. In this study, we investigated the extent to which different face masks alter speech intelligibility in individuals with different degrees of hearing impairment. Methods: A total of fifty participants were divided into four hearing status groups according to the degree of hearing loss: normal levels (16), mild (13), moderate (11), and severe (10). A modified version of the Audiovisual German Matrix Sentence Test (AV-OLSA) was used to assess speech perception in noise in five conditions (audiovisual, audio-only, visual-only, surgical mask, and FFP2 mask). Results: Our results show that acoustic attenuations of face masks cause a small but similar decrease in speech reception thresholds (SRTs) in listeners of different hearing statuses. The effect of visual cues (visual benefit) on SRTs was stronger than the effect of acoustic attenuation but also did not differ significantly between the different hearing status groups, with a median difference of 1.5 dB for mild hearing loss, 2.9 dB for moderate hearing loss, and 2.7 dB for severe hearing loss. The best-aided hearing status did not correlate with visual benefit. Conclusions: Our research confirms the importance of providing visual cues for speech reception in noisy environments, especially for individuals with impaired hearing, regardless of their degree of hearing loss. Full article

Background/Objectives: The OTOG gene is responsible for autosomal recessive non-syndromic sensorineural hearing loss and is assigned as DFNB18B. To date, 44 causative OTOG variants have been reported to cause non-syndromic hearing loss. However, the detailed clinical features for OTOG-associated hearing loss remain unclear. Methods: In this study, we analyzed 7065 patients with non-syndromic hearing loss (mean age 26.4 ± 22.9 years, 2988 male, 3855 female, and 222 without gender information) using massively parallel DNA sequencing for 158 target deafness genes. We identified the patients with biallelic OTOG variants and summarized the clinical characteristics. Results: Among the 7065 patients, we identified 14 possibly disease-causing OTOG variants in 26 probands, with 13 of the 14 variants regarded as novel. Patients with OTOG-associated hearing loss mostly showed congenital or childhood-onset hearing loss. They were considered to show non-progressive, mild-to-moderate hearing loss. There were no symptoms that accompanied the hearing loss in OTOG-associated hearing loss patients. Conclusions: We confirmed non-progressive, mild-to-moderate hearing loss as the clinical characteristics of OTOG-associated hearing loss. These findings will contribute to a better understanding of the clinical features of OTOG-associated HL and will be useful in clinical practice. Full article

Background: Hearing loss (HL) among older adults is a major global health concern. Hearing aids (HAs) offer an effective solution to manage HL and enhance the quality of life. However, the adoption and the consistent use of HAs remain low, making non-use a significant barrier to successful audiological rehabilitation. The aim of the study was to assess the benefit of HAs among patients with different degrees of HL and to determine the profiles of patients who have the least benefit from HAs. Methods: the HA benefits were assessed by using the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire. Participants were assigned to the study groups based on the pure-tone audiometry. This paper presents the results obtained by using HAs in various listening environments among 167 patients. Results: The majority of individuals benefited from HAs in a noisy environment while a reverberant environment provided the lowest benefit. It was observed that the degree of HL had a statistically significant impact on the benefits of HAs in terms of the communication ease, the reverberation, the background noise, and the global score. A moderately positive correlation was observed between the unaided APHAB and the HL degree. The subjects’ APHAB scores ranged from the 50th to the 65th percentile. Additionally, women had a significantly better improvement than men. Conclusions: HAs improved communication in everyday life situations among 91.6% of HA users. The degree of HL influences APHAB scores. Patients with a severe degree of HL achieved the greatest APHAB scores while male patients with mild HL received the lowest benefits of HAs. Both HL and the age, gender, and HA type are factors that also play important roles. The APHAB questionnaire is a reliable screening test for patients with hearing difficulties. Full article

Background/Objectives: The present study investigates the reasons for better recognition of disyllabic words in Malayalam among individuals with hearing loss. This research was conducted in three experiments. Experiment 1 measured the psychometric properties (slope, intercept, and maximum scores) of disyllabic wordlists. Experiment 2 examined PB_max scores across varying degrees of sensorineural hearing loss (SNHL) and compared these findings with studies in other Indian and global languages. Experiment 3 analyzed the recognition performance of different vowel combinations across varying degrees of hearing loss. Methods: Experiment 1: Psychometric functions for disyllabic word recognition were derived from 45 individuals with normal hearing. Word recognition was tested in quiet at nine hearing levels ranging from −10 to +40 dB HL. Experiment 2: 1000 participants with SNHL were categorized by hearing loss severity (mild, moderate, moderately severe, severe, and profound). Word recognition scores, including PB_max, were analyzed and compared across severity levels. Experiment 3: Percent error scores for 17 vowel combinations were assessed in 37 participants with SNHL. Ten disyllabic words represented each combination. Results: Disyllabic wordlists showed significantly higher word recognition scores than monosyllabic lists across all degrees of hearing loss. Individuals with mild-to-moderately severe SNHL achieved higher PB_max scores, with performance declining at severe- and profound-loss levels. The higher recognition of disyllabic words was attributed to contextual cues and low-frequency vowel-based information, particularly benefiting those with residual low-frequency hearing. Error analysis highlighted the influence of specific vowel combinations on word recognition performance. Conclusions: Disyllabic words are easier to recognize than monosyllabic words for individuals with SNHL due to their rich contextual and low-frequency energy cues. Disyllabic wordlists sustain higher recognition scores up to moderately severe hearing loss but show a marked decline with more severe losses. The phonemic balance of wordlists and vowel combinations significantly influences word recognition, emphasizing the importance of these factors in developing wordlists for clinical use. Full article

Treacher Collins syndrome (TCS) is a rare congenital craniofacial disorder with variable penetrance and high genetic and phenotypic heterogeneity. It is caused by pathogenic variants in the TCOF1, POLR1D, POLR1C, and POLR1B genes, and its major characteristic features are malar and mandibular hypoplasia, downward slanting of the palpebral fissures, and conductive hearing loss. In this study, five patients (two males and three females, age range from 2 to 29 years) with TCS were tested by Next-Generation Sequencing (NGS)-based sequencing and clinically characterized. Genetic analyses detected two deletions and one insertion in the TCOF1 gene and one missense variant in the POLR1D gene. Two novel mutations, c.1371_1372insT (p.Lys458*) in the TCOF1 gene and c.295 G>C (p.Gly99Arg) in the POLR1D gene, were identified. Moreover, two already known mutations, c.4369_4373del (p.Lys1457Glufs*12) and c.2103_2106del (p.Ser701Argfs*9) in the TCOF1 gene, were detected. The novel TCOF1 c.1371_1372insT mutation was associated with mild craniofacial manifestations and very rare symptoms of TCS, i.e., developmental delay and moderate intellectual disability. Although incomplete penetrance is a known phenomenon in TCS, surprisingly, the majority of our patients inherited the disease-causing variants from an asymptomatic mother. The unique feature of our study is the observation of causative mutation transmission between asymptomatic family members. Our results expanded the clinical and mutational spectrum of TCS and further confirmed the inter- and intra-familial variability of this disorder. Full article

Background and Objectives: This study aimed to investigate the association between insulin resistance, as measured by the triglyceride–glucose index (TyG index), and hearing impairment in middle-aged women in Korea. Materials and Methods: This cross-sectional survey utilized data from the Korea National Health and Nutrition Examination Survey (KNHANES) IV (2007–2009), specifically from the period after July 21, 2009, when hearing test results became available, and from the KNHANES V (2010–2012). This study was conducted on 5416 women aged 40 to 69 who had completed both the health examination survey and audiometric tests, excluding those with missing data on menopausal status and the use of hormone replacement therapy. Results: In the study group, the prevalence of high-frequency hearing loss according to the TyG index was significantly higher in the mild hearing loss group (OR = 1.29; 95% CI: 1.12, 1.49, p < 0.001) and the moderate hearing loss group (OR = 1.27; 95% CI: 1.09, 1.48, p = 0.002). Conversely, the prevalence of low-frequency hearing loss did not show a significant difference in either the mild hearing loss group (OR = 1.17; 95% CI: 0.99, 1.37, p = 0.065) and the moderate hearing loss group (OR = 1.13; 95% CI: 0.94, 1.35, p = 0.199) Conclusions: Since diabetes can induce hearing impairment in women, it is recommended that women with a high TyG index undergo early hearing tests Full article

Background: Speech recognition in noise is one of the difficulties faced by people with hearing loss that increases with age. The recently developed Spanish Auditory Test of Speech in Noise (Prueba Auditiva de Habla en Ruido en Español, PAHRE) allows for the identification of these intelligibility difficulties in noise. The aim of this study was to assess speech recognition in noise in people with hearing loss and to test the benefits of the Lombard effect. Methods: The participants were 104 people with hearing difficulties, ranging in age from 37 to 98 years. The variables age, degree of hearing loss, presence of high-frequency dropout, and years of formal education were assessed. Results: Psychometric curves were obtained as a function of signal-to-noise ratio as well as threshold values of speech reception as a function of age group for mild and moderate hearing loss. The results indicated that the speech reception threshold increased with both age and the degree of hearing loss, becoming particularly significant after the age of 70. Furthermore, it was found that the combined factors of age, degree of hearing loss, and educational level predicted a high percentage of the variability in the speech reception threshold. Conclusions: Therefore, the Spanish Auditory Test of Speech in Noise could be a useful clinical tool for diagnosis, monitoring, auditory rehabilitation planning, and hearing aid fitting. Full article

Over-the-counter hearing aids (OTC HAs) have the potential to help adults with perceived mild-to-moderate hearing loss across the US, especially in rural communities, where access to hearing healthcare is extremely limited or non-existent. The purpose of this study was to describe an OTC HA clinical trial being conducted in five rural counties of Alabama and to provide preliminary anecdotal data related to the use and care of these hearing aids by the participants. In brief, for this clinical trial, adults with hearing loss were randomly placed in one of three groups where they received varying levels of support for setting, using, and maintaining their OTC HAs. Listening tests and surveys were administered to assess the extent to which they benefitted from the hearing aids as related to word understanding, communication with others in natural settings, and hearing aid use and care. Currently, anecdotal findings suggested that, although some participants required very little support to successfully use their hearing aids, others had difficulty setting and caring for their devices and could have benefitted from individualized guidance. Future quantitative studies will assess the extent of support needed for successful hearing aid benefit and use. Potentially, collaborations among pharmacy and audiology professionals could lead to increased access to hearing healthcare by supporting the use and purchase of OTC HAs in rural pharmacy settings. Full article

Congenital cytomegalovirus (CMV) infection is the main cause of non-hereditary sensorineural hearing loss (SNHL). In order to shed light on SNHL pathophysiology, we examined the auditory pathway in CMV-infected fetuses; the temporal lobe, in particular the auditory cortex, and the inner ear. We investigated both inner ears and temporal lobes of 20 human CMV-infected fetuses at 21 weeks of gestation. As a negative group, five fetuses from spontaneous miscarriages without CMV infection were studied. Inner ears and temporal lobes were histologically examined, immunohistochemistry for CMV and CMV-PCR were performed. On the auditory cortex, we evaluated the local microglial reaction to the infection. CMV-positive cells were found in 14/20 brains and the damage was classified as severe, moderate, or mild, according to histological features. Fetuses with severe brain damage had a statistically higher temporal lobe viral load and a higher number of activated microglial cells in the auditory cortex compared to fetuses with mild brain damage (p: 0.01; p: 0.01). In the inner ears, the marginal cells of the stria vascularis were the most CMV positive. In our study, CMV affected the auditory pathway, suggesting a tropism for this route. In addition, in the auditory cortex, microglial activation may favor further tissue damage contributing to hearing loss. Full article

The aim of the present study was to investigate adverse effects of head injury, neck trauma, and chronic noise exposure on the complaint profile in people with Ménière’s disease (MD). The study used a retrospective design. Register data of 912 patients with MD from the Finnish Ménière Federation database were studied. The data comprised case histories of traumatic brain injury (TBI), neck trauma and occupational noise exposure, MD specific complaints, impact related questions, and the E-Qol health-related quality of life instrument. TBI was classified based on mild, moderate, and severe categories of transient loss of consciousness (TLoC). The mean age of the participants was 60.2 years, the mean duration of the disease was 12.6 years, and 78.7% were females. Logistic regression analysis, linear correlation, and pairwise comparisons were used in evaluating the associations. 19.2% of the participants with MD had a history of TBI. The phenotype of participants with TBI was associated with frequent vestibular drop attacks (VDA), presyncope, headache-associated vertigo, and a reduction in the E-QoL. Logistic regression analysis explained the variability of mild TBI in 6.8%. A history of neck trauma was present in 10.8% of the participants. Neck trauma associated with vertigo (NTwV) was seen in 47 and not associated with vertigo in 52 participants. The phenotype of NTwV was associated with balance problems, VDA, physical strain-induced vertigo, and hyperacusia. Logistic regression analysis explained 8.7% of the variability of the complaint profile. Occupational noise exposure was recorded in 25.4% of the participants and correlated with the greater impact of tinnitus, hyperacusis, and hearing loss. Neither the frequency, duration, or severity of vertigo or nausea were significantly different between the baseline group and the TBI, NTwV, or noise-exposure groups. The results indicate that TBI and NTwV are common among MD patients and may cause a confounder effect. Full article

This patient case report describes a first experience in late 2022 and early 2023 with over-the-counter (OTC) hearing aids for a 71-year-old male with self-perceived, age-related hearing loss. The patient reported no “red flag” medical conditions that would preclude him from safely using an OTC hearing aid device. After also meeting inclusionary criteria required to be printed on the device label, the patient was offered FDA registered OTC hearing aids. The first device pair was returned due to malfunction. The second device pair was an in-the-canal style, black in color, and powered by disposable batteries. He required help setting up the device from his spouse, an audiologist, and a pharmacist. Improved scores on the Self-Assessment of Communication and Significant Other Assessment of Communication were noted from the patient and his spouse. The patient continued to use the second device pair for 6 months after first use with no additional help. Our experience supports the pharmacist’s role in identifying appropriate candidates for OTC hearing aids, helping patients select a device, and supporting device setup and self-fitting processes at community pharmacies. Further experiences are needed to demonstrate how pharmacists can support OTC hearing aid purchases at community pharmacies. Full article

Inner ear involvement (IED) is a rare local complication of the very common acute otitis media (AOM). The most beneficial treatment for IED remains a matter of debate. The aim of this study is to analyze different treatment modalities based on hearing outcomes to contribute to the discussion of therapy for IED in AOM. This retrospective study includes 112 adult patients diagnosed with AOM with IED between 2000 and 2020. Patients either received conservative (systemic antibiotic and systemic steroid therapy), interventional (conservative plus myringotomy and tympanic tube) or operative (interventional plus antrotomy) treatment. Pre- and post-treatment pure tone audiometry was performed. The hearing outcome was compared, and hearing recovery was analyzed based on modified Siegel’s criteria. The pre-treatment pure tone average (PTA) was significantly (p < 0.05) higher in the operative group than in the other groups. All treatment modalities led to a significant hearing improvement (p < 0.001). The pre- and post-treatment hearing loss was predominantly observed in high frequencies 2–4 kHz. The operative group showed the highest rate of complete hearing recovery. While all treatment modalities led to a significant improvement in hearing, the operative group showed the most beneficial hearing results in patients with high pre-treatment hearing loss. It remains to be shown if the findings in patients with high pre-treatment hearing loss can be generalized to patients with mild or moderate pre-treatment hearing loss. Full article

Non-syndromic hearing impairment (NSHI) is a very heterogeneous genetic condition, involving over 130 genes. Mutations in GJB2, encoding connexin-26, are a major cause of NSHI (the DFNB1 type), but few other genes have significant epidemiological contributions. Mutations in the STRC gene result in the DFNB16 type of autosomal recessive NSHI, a common cause of moderate hearing loss. STRC is located in a tandem duplicated region that includes the STRCP1 pseudogene, and so it is prone to rearrangements causing structural variations. Firstly, we screened a cohort of 122 Spanish familial cases of non-DFNB1 NSHI with at least two affected siblings and unaffected parents, and with different degrees of hearing loss (mild to profound). Secondly, we screened a cohort of 64 Spanish sporadic non-DFNB1 cases, and a cohort of 35 Argentinean non-DFNB1 cases, all of them with moderate hearing loss. Amplification of marker D15S784, massively parallel DNA sequencing, multiplex ligation-dependent probe amplification and long-range gene-specific PCR followed by Sanger sequencing were used to search and confirm single-nucleotide variants (SNVs) and deletions involving STRC. Causative variants were found in 13 Spanish familial cases (10.7%), 5 Spanish simplex cases (7.8%) and 2 Argentinean cases (5.7%). In all, 34 deleted alleles and 6 SNVs, 5 of which are novel. All affected subjects had moderate hearing impairment. Our results further support this strong genotype–phenotype correlation and highlight the significant contribution of STRC mutations to moderate NSHI in the Spanish population. Full article