Moderate Hearing Impairment Research Articles

The effect of aging and asymmetrical hearing on speech discrimination

BackgroundSpeech discrimination (SpD) is exacerbated by hearing loss, but the relationship between pure-tone audiometry (PTA) and SpD is poorly described and understood in the aging process. It is also unclear whether severity of left-right (L-R) differences in PTA threshold disproportionately affects SpD.MethodsWe conducted a retrospective cross-sectional study of a broad age range (10–99 years) of patients in Japan. Demographic data, SpD, and PTA threshold data were collected for each patient and side (L-R). We evaluated the association between chronological age and overall SpD, and SpD according to severity of PTA-threshold difference for ear pairs. Asymmetries in L-R PTA thresholds were stratified into four equally spaced categories: <10dB L-R difference to ≥40 dB L-R difference.ResultsAmong the 2760 eligible participants, data from 5508 ears are analyzed. Overall SpD gradually decreases with age, with SpD scores in the oldest groups being significantly worse (Kruskal-Wallis followed by Dunn’s test; p < 0.0001) than those of the youngest group. Comparison of worse-hearing ears to better-hearing ears within the same severity of hearing impairment reveals significant differences on SpD in ears that have moderate or severe hearing impairment (p < 0.0001). Post-hoc analysis identifies significant differences in SpD between the worse-hearing ears and the better-hearing ears that have hearing differences of 20 dB or more.ConclusionsSpD is dramatically affected by age and asymmetrical hearing. Therefore, appropriate audiological interventions should be proactively considered according to an individual’s hearing level, age, and difference in L-R hearing levels.

External auditory canal cholesteatoma in children: clinical manifestations.

The purpose of this study was to explore the characteristics of external auditory canal cholesteatoma (EACC) among children and to describe its radiological findings on high-resolution computed tomography (CT) of the temporal bone in order to improve the diagnostic accuracy of primary EACC. The clinical records and CT imaging features of 44 patients who were diagnosed with EACC between January 2017 and May 2022 at Shenzhen Children's Hospital were retrospectively reviewed. Clinical features, including external auditory canal wall findings, hearing damage, symptoms and physical examination findings, were analysed against the level of lesion involvement. The correlation between different types of EACC and the incidence of different clinical symptoms was analysed, and the degree of hearing impairment and the rate of bone wall destruction were examined using CT. The mean age at EACC onset was 9.02 ± 3.15 years, and the mean age at onset for EACC involving the right ear was older than that of EACC involving the left ear (P < 0.05). There were 44 patients (46 ears), including 10 ears with type I EACC, 23 ears with type II EACC, and 13 ears with type III EACC. Conductive hearing loss was the main type of hearing impairment observed among EACC patients. There were differences in types I, II and III EACC in terms of hearing impairment; specifically, there was a significant difference in moderate hearing impairment between type II and type III EACC patients (P < 0.05). The four most common symptoms were otorrhea, otalgia, itching and bleeding. The incidence of itching symptoms was greater in type I EACC than the incidence of otorrhea, and the incidence of otorrhea symptoms in type II and type III EACC was significantly greater than that in type I EACC(P < 0.05). There were no significant differences in the fracture rates of the anterior, posterior, superior or inferior walls of the external auditory canal within or between type II and type III EACC patients (P > 0.05). The failure rate of scute damage was significantly higher in type III EACC patients than in type II EACC patients (P < 0.05). The presence of otorrhea and hearing loss as well as the identification of granulation tissue during otoscopy suggest the need for a temporal bone CT scan. This imaging modality can aid in the early detection and accurate classification of EACC, thereby guiding the selection of appropriate surgical interventions and greatly assisting in preventing further progression of hearing impairment.

Hearing Impairment and Physical Activity and Physical Functioning in Older Adults: Baseline Results From the ACHIEVE Trial.

Hearing loss is associated with restricted physical activity (PA) and impaired physical functioning, yet the relationship between severity of hearing impairment (HI) and novel PA measures in older adults with untreated HI is not well understood. Analyses included 845 participants aged ≥70 years (mean = 76.6 years) with a better-hearing ear pure-tone average (PTA) ≥30 and <70 dB in the Aging and Cognitive Health Evaluation in Elders (ACHIEVE) study who wore an ActiGraph accelerometer for 7 days. Physical functioning measures included grip strength and the Short Physical Performance Battery (SPPB). Linear regression models estimated the association by HI level (moderate or greater [PTA ≥ 40 dB] vs mild [PTA < 40 dB]) and continuous hearing with total daily activity counts, active minutes/day, activity fragmentation, grip strength, and gait speed. Logistic regression models estimated odds ratios (ORs) and 95% confidence intervals (CIs) of poor performance on the SPPB (≤6) and its subtests (≤2). Mixed-effects models estimated differences by HI level in activity by time of day. Participants with moderate or greater HI had poorer physical functioning, particularly balance (OR = 2.17, 95% CI = 1.29-3.67), versus those with mild impairment. There was no association of HI level with activity quantities or fragmentation. For diurnal patterns of activity, participants with moderate or greater HI had fewer activity counts in the afternoon (12:00 pm -05:59 pm). Older adults with worse hearing had shifted diurnal patterns and poorer balance performance. Exercise programs should be tailored to older adults with different levels of HI to maintain PA and physical functioning, particularly balance control.

Inquiring Life span Impact of Mild and Moderate Hearing Impairment on Diverse Aspects of Development

The current study was particularly designed to endeavor the effects of hearing impairment on the developmental dimensionsof persons with hearing impairment. Mild and moderate hearing impairment was assumed to be as independent factor having mild and moderate levels of losses. Multidimensional developmental dimensionsof persons with hearing impairment were taken to be as dependent variables such as cognition, physical, moral, social as well as economicdevelopments. The parents as well as relatives of the persons with hearing impairment from Faisalabad, Jhang and Chiniot citieswere chosen as the population of the study while sample was comprised ofn=208 participantschosen through purposive sampling technique. Effects were stronger among older n=51 with mean ages 69 years (65-74 years) than youngern=72 with mean ages 36 years (20-44 years) and middle aged n=77 with mean ages 58 years (45-64 years) manifesting long lasting effects of independent variable (hearing impairment) on dependent variables (developmental dimensions) of persons with mild and moderate hearing losses. The results of the study revealed monotonically increasing function of developmental dimensions that is affected by the level of disability as in the case of hearing impairment, the effects of disability on the person’s developmental dimensions always increase or remain constant and never decrease in any case or in any circumstances at any stage of life.

Novel Pathogenic Variants in the Gene Encoding Stereocilin (STRC) Causing Non-Syndromic Moderate Hearing Loss in Spanish and Argentinean Subjects.

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.

Facial and Cochlear Nerves Outcomes in the Surgical Resection of Giant Vestibular Schwannoma: Is There Any Predictive Value for Intraoperative Neuromonitoring of the Facial Nerve?

Background: Giant vestibular schwannoma (VS) represents one of the most challenging interventions in skull base surgery. Preserving facial and cochlear nerves requires effective surgical planning and nuanced techniques. Objectives: The present study evaluates the role of intraoperative neuromonitoring (IONM) in predicting the outcome of facial and cochlear nerves. Methods: This retrospective cohort comprised 34 patients with a giant (Samii IV/IVb grade) VS during 2016 - 2019. The retrosigmoid approach was used for tumor resection. Pre- and postoperative facial exams were graded according to the House-Brackmann Scale. Hearing ability was also classified according to the Hannover Hearing Classification. Intraoperative findings included the location of the nerve complex, tumor consistency, the surgical plane of the tumor from the facial nerve/ brainstem, and the level of internal auditory canal (IAC) decompression. Intraoperative neuromonitoring was used for all surgeries. The amplitude required for a positive response was recorded during the facial nerve's direct electrical stimulation (DES). All patients were followed 6 and 12 months postoperative. Results: The preoperative facial nerve function was normal in 91.2% of patients, and 8.8% had slight facial nerve impairment (FNI). Considering preoperative hearing status, 9 cases (26.5%) had moderate hearing loss or impairment, while 25 patients (73.5%) complained of severe hearing impairment. The mean intraoperative direct facial nerve stimulation threshold was 1.38 ± 0.89. Hearing impairment was moderately severe in 7 (20.6%) and severe in 25 patients (73.5%) postoperation. In addition, postoperative assessment revealed 13 (38.2%) cases with moderate FNI and 12 (35.2%) with moderately severe FNI. The tumor size did not affect the postoperative hearing loss, and the postoperative hearing loss did not improve significantly (P = 0.32). There was no statistically significant correlation between the intensity of intraoperative DES and postoperative facial nerve function (P &gt; 0.05). No significant correlation was observed between tumor consistency and postoperative hearing status (P &gt; 0.05). Conclusions: The retrosigmoid approach combined with IONM appears to be associated with favorable facial and cochlear nerve outcomes in giant VS. The postoperative facial nerve function may improve, but sensorineural hearing status may not improve significantly. Moreover, the intensity of intraoperative DES may not predict the postoperative facial function. Therefore, the findings of IONM should be interpreted carefully.

Factors associated with balance impairments in the community-dwelling elderly in urban China

BackgroundIdentification of factors relevant to balance performance impairments in the elderly population was critical for developing effective interventions and preventions. However, there have been very limited data available based on large scale studies. The present study identified factors that independently contributed to performance impairments in overall balance, domains of static balance, postural stability, and dynamic balance, and individual items.MethodsA total of 1984 community-dwelling Chinese elderly from urban areas of Shanghai were recruited. Information on demographic characteristic, exercise, and health status were collected with a face-to-face interview. Balance performances were assessed on site by trained investigators based on the X16 balance testing scale. To identify the effectors, ordinal logistic regression analysis was applied for overall balance, static balance, postural stability, and dynamic balance. Binary logistic regression analysis was used for 16 items.ResultsThe community-dwelling elderly residents were aged from 60 to 97 years old. With increases of age, risks of impairments in overall balance increased gradually (ORs from 1.26 to 3.20, all P < 0.01). In the elderly with overweight and obesity, there was higher proportion of balance impairments compared to the elderly with normal BMI (OR = 1.26, P < 0.001). Regular exercise every week was associated with reduced risks of balance impairments (ORs from 0.63 to 0.73, all P < 0.001). Presences with vision lesion (ORs from 1.28 to 1.59, all P < 0.001), moderate hearing impairment (OR = 1.54, P < 0.001), somesthesis dysfunction (ORs from 1.59 to 13.26, all P < 0.001), and cerebrovascular disease (OR = 1.45, P = 0.001) were related to increased risks of balance impairments. Likewise, age, exercise, vision, hearing, somesthesis, and cerebrovascular disease were significantly associated with static balance, postural stability, and dynamic balance. Both overweight and obesity and underweight were associated with higher proportions of dynamic balance impairments. Regular exercise was significantly related to reduced risks of impairments in 15 out of the 16 items.ConclusionsIn the elderly, age, overweight and obesity, exercise, vision, hearing, somesthesia, and cerebrovascular disease were dominant factors associated with impairments in overall balance, domains of static balance, postural stability, and dynamic balance, and most individual items.Trial registrationNot applicable.

ON THE QUESTION OF HEARING IMPAIRMENT IN PREMATURE CHILDREN WHO HAVE EXPERIENCED OXIDATIVE STRESS AT BIRTH

Introduction. Hearing impairment (HI) occupies an important place among perinatal pathology. According to theWHO, the frequency of severe hearing impairment is observed in 1–2 per 1000 newborns and in 15% of newborns whorequired intensive therapy after birth. Mild and moderate HI occurs in 1–2% of children with perinatal pathology.The aim of the work is to study the predictors of hearing impairment and the factors that determine the severityof deafness in children aged 2–3 months who suffered oxidative stress at birth.Material and methods. 131 cases of observation of newborns of 27–36 weeks of gestation with HI in Communalnonprofit enterprise «City perinatal center» Kharkiv city council were analyzed. Children whose families had casesof HI were excluded from the study. During the observation, the audiological examination was carried out in thenewborn period (primary examination) and at 2–3 months of age. To meet the aim of the work, alternative groupswere formed: Group I (n=51) with unilateral and Group II (n=80) with bilateral hearing impairment.To determine the predictors of the severity of PS in the comparison groups, the factors determining deafnesswere studied, and their prognostic informativeness was established. The t-Student and φ-Fisher criteria weredetermined, the Wald-Henkin heterogeneous sequential procedure algorithm was used, predictive coefficients (PC)and informativeness (I) were determined.By decision of the Bioethics Commission № 3 dated March 21, 2023, the study materials comply with the TokyoDeclaration of the World Medical Association, the International Recommendations of the Helsinki Declaration onHuman Rights, the Council of Europe Convention on Human Rights and Biomedicine, the Laws of Ukraine, and therequirements of the Code of Ethics of a Doctor of Ukraine.The study was carried out within the framework of the scientific research work of the Department of Pediatrics№ 3 and Neonatology of KhNMU «Study of peculiarities of the course of oxidant stress diseases in newborns» 2022–2024 (state registration № А22U000025).Results. Factors of obstetric and somatic anamnesis of the mother did not influence the nature of HI in the child.Among the complications of pregnancy and childbirth in the anamnesis of children with bilateral HI, dysfunction ofplacenta was more often observed 33.8 and 13.7 % (р&lt;0.001), threat of abortion 30.0 and 17.6 % (&lt;0.05), anemiapregnant women 31.2 and 13.7 % (p&lt;0.001), fetal distress 56.2 and 37.3 % (p&lt;0.01), cesarean delivery 68.8 and 47.1% (p &lt;0.001), especially regarding fetal distress – 67.3 and 29.2 % (р&lt;0.001).The frequency of bilateral HI in children is inversely proportional to gestational age and birth weight and isassociated with a low Apgar score. Sexual dimorphism was revealed: girls predominated in the group with bilateralhearing loss, 56.2 and 35.3 % (р&lt;0.001). Children with bilateral HI more often required surfactant therapy 78.8 and33.3 % (p&lt;0.001), as well as longer respiratory support and oxygen therapy.In the study, bilateral HI was more common in children with diseases whose pathogenesis is closely relatedto oxidative stress: periventricular leukomalacia 55.0 and 7.8 % (&lt;0.001); bronchopulmonary dysplasia (BPD)of the 2nd and 3rd degree: 23.7 and 2.0 % (p&lt;0.001) and 23.7 and 0 % (p&lt;0.001), respectively; retinopathy ofprematurity grade 2–3 42.5 and 17.6 % (р&lt;0.001); with an PDA 81.3 and 41.2 % (p&lt;0.001), including when surgicalclosure was necessary, 18.5 and 9.5 % (p&lt;0,05). The highest informativeness of hearing impairment in childrenwho have undergone oxidative stress belongs to the following characteristics: gestational age (6.14), total durationof mechanical ventilation (5.54), body weight (5.44), BPD (5.4), duration of mechanical ventilation in mode ofnormoventilation (4.1), duration of oxygen therapy (3.91), PDA (3.72).The most significant prognostic factors of bilateral hearing impairment: the presence of BPD 2–3 stages. (-13.8);intraventricular hemorrhage (IVH) 2–3 st. (-12.4); total duration of ventilator ≥21 days (-10.33); gestation period≤29 weeks (-8.6); periventricular leukomalacia (PVL) 2–3 st. (-8.48); duration of oxygen therapy ≥21 days (-7.38);body weight (-6.94).Conclusions: The most significant prognostic factors of bilateral hearing impairment: the presence of BPD 2–3st. (-13.8); IVH 2–3 st. (-12.4); total duration of ventilator ≥21 days (-10.33); gestation period ≤29 weeks (-8.6);periventricular leukomalacia 2–3 st. (-8.48); duration of oxygen therapy ≥21 days (-7.38); body weight (-6.94), etc.Bilateral hearing impairment is predicted in newborns based on the presence of gestational immaturity (gestationalterm ≤ 29 weeks), BPD grade 2–3, IVH grade 2–3, total duration of mechanical ventilation and oxygen therapy &gt; 21days, PVL grade 2–3. In our study, no reliable relationship was found between the nature of the hearing impairment of aprematurely born child and the somatic and obstetric anamnesis of the mother. Gender characteristics among prematureinfants with bilateral HI were characterized by the predominance of the female gender, 56.2 and 35.3 % (р&lt;0.001).

Population Based Study and Global Estimates of Hearing Impairment in Schoolchildren

Hearing impairments in schoolchildren affects learning and communication. Reliable data on hearing loss prevalence in this group are necessary for planning the audiological care. Aim. To compare data from a population based study of the hearing loss prevalence in schoolchildren and estimates from the Global Burden of Disease (GBD) study in the Republic of Yemen. Materials and methods. Data extraction was performed from the GBD database on the prevalence of bilateral hearing loss &gt;20 dB among children aged 5–9 years in the Republic of Yemen. A two stage study of hearing loss prevalence among primary school students aged 6–9 years in Sana’a, Republic of Yemen, in a sample of 2200 children using screening audiometry at 20 dB at 0.5, 1, 2, and 4 kHz is school settings, followed by tonal threshold audiometry and tympanometry in a specialized clinic. Hearing loss was assessed as unilateral or bilateral when hearing thresholds were higher than 25 dB. Results. According to GBD estimates, bilateral hearing impairment in this population is 2.2%, including 1.4% of mild degree. According to the results of a Yemenian population study, mild and moderate hearing impairment was detected in 10.6%, of which 6.8% – unilateral, 3.8% – bilateral hearing loss. There were no cases of severe or profound hearing loss. The prevalence of otitis media with effusion was 6%, chronic suppurative otitis media – 1.8%, sensorineural hearing loss – 1.6%. Conclusion. Bilateral hearing impairments occur in 2.2–3.8% of primary school students, with unilateral impairments the prevalence is up to 10.6%. Of these, at least 65% have conductive hearing loss due to pathology of the middle ear. The data obtained will improve the efficiency of ear and hearing care for schoolchildren.

Prevalence of moderate or greater permanent childhood hearing impairment and effectiveness of targeted surveillance for babies who pass newborn hearing screening

Objective To assess the utility of targeted surveillance for the identification of moderate to profound PCHI in babies who pass newborn hearing screening in England and have risk factors. Design Retrospective analysis. Study sample A total of 3,957,891 children born 01/04/2012–31/03/2018 in England. Results A total of 7148 PCHI cases were identified (1.81 per 1,000 babies). 6,707 followed an immediate referral from the screen (1 per 16 referrals), 51 followed targeted surveillance referral (1 per 540 referrals) and 390 without a referral. Audiology uptake was higher following an immediate referral (96.7% overall, 77.2% within NHSP-defined timescales) than following targeted surveillance (63.8% overall, 51.1% within 52 weeks of birth). The screening was 94.5% sensitive overall, with similar sensitivities for each of the risk factors. General linearised logistic regression models identified syndrome as the risk factor with the highest odds ratio (14.08 for all babies, 22.19 for babies without immediate referral). Close family history of hearing loss was the next highest (10.93 for all babies, 12.29 for babies without immediate referral). Conclusion The evidence for a targeted surveillance programme, based on risk factors, for babies in England who pass the newborn screen is not strong.

Factors Leading to Conductive Hearing Loss among Children Living in Delhi Slum Areas

Background: Middle-ear diseases in children pose a significant burden to the healthcare system. Recurrent upper respiratory tract infections, cold, and cough often initiate middle ear disease. It results in mild to moderate hearing impairment that can be detrimental to the child's linguistic development. It can further lead to behavioural and cognitive problems. The study was designed to establish a relationship between common middle pathologies like CSOM and OME with hearing loss in children.&#x0D; Objective: To determine the prevalence of middle ear pathology and its association with hearing loss in slum children of Delhi.&#x0D; Methods: A study was conducted with 269 children aged five to ten years. The children were screened by otoscopic examination, followed by Tympanometry and then by Pure Tone Audiometry.&#x0D; Results: The prevalence of middle ear problems was 24.2%, out of which the prevalence of Chronic Suppurative Otitis Media (CSOM) was 10.8%, Otitis media with effusion (OME) was 12.3% and Acute Otitis Media (AOM) was 1.1%. A significant correlation (p&lt; 0.005) was found between hearing loss and middle ear pathology like CSOM and OME. Out of the 65 children with ear pathology, hearing loss was detected in 55 children, out of which 29 children with CSOM, moderated conductive hearing loss was detected in 28 children and out of 33 OME children, mild to moderate conductive hearing loss was detected in 26 children.&#x0D; Conclusion: The article highlights the high prevalence of middle ear pathology in underprivileged children. The study also concludes the high probability of conductive hearing loss in these children. It is recommended that there is more need for awareness for early detection and treatment of middle ear infection to avoid any language and behavioural problems in the future. Also, the disparity between children living in urban and rural areas needs to be bridged.

Evaluation of the Association Between Hearing Aids and Reduced Cognitive Decline in Older Adults with Hearing Impairment.

We investigated whether hearing aids (HAs) for hearing impairment may reduce cognitive decline in older adults. Retrospective cohort. Ambulatory for medical checkup. Comprised 1,453 older adults aged 65 to 90 years. HAs were recommended for hearing impairment with thresholds of ≥40 dB. The Mini-Mental State Examination (MMSE) and pure-tone audiometry were performed. Over 2 years, multivariate logistic regression analysis was used to assess the effects of sex, age, hearing, HAs, hypertension, diabetes, cerebrovascular disease, smoking, and drinking on MMSE scores. Of 1,453 participants who underwent MMSE, 1,320 who underwent audiometry in the first-year included 99 HA users. Of the 1,320 participants, 858 (89 HA users) were followed longitudinally for 1 year and 672 (66 HA users) for 2 years. In the first-year cross-sectional study, the risk ratios (RRs) for hearing impairment (≥25 dB) when the MMSE scores were ≤23 and ≤27 points were 2.97 (1.40-6.28) and 1.34 (1.01-1.79), respectively. For moderate (40-<70 dB) and moderate to severe (≥40 dB) hearing impairment, the RRs for HA use were 0.30 (0.11-0.86) and 0.50 (0.22-1.13), respectively, when the MMSE scores were ≤23. A 2-year follow-up of participants with moderate hearing impairment yielded hazard ratios for MMSE score decrease of 0.30 (0.11-0.82) after 1 year and 0.16 (0.04-0.64) after 2 years in HA users. HA use may be associated with reducing MMSE score decrease in older adults with moderate hearing impairment.

The phenotypic spectrum of terminal and subterminal 6p deletions based on a social media-derived cohort and literature review

BackgroundTerminal 6p deletions are rare, and information on their clinical consequences is scarce, which impedes optimal management and follow-up by clinicians. The parent-driven Chromosome 6 Project collaborates with families of affected children worldwide to better understand the clinical effects of chromosome 6 aberrations and to support clinical guidance. A microarray report is required for participation, and detailed phenotype information is collected directly from parents through a multilingual web-based questionnaire. Information collected from parents is then combined with case data from literature reports. Here, we present our findings on 13 newly identified patients and 46 literature cases with genotypically well-characterised terminal and subterminal 6p deletions. We provide phenotype descriptions for both the whole group and for subgroups based on deletion size and HI gene content.ResultsThe total group shared a common phenotype characterised by ocular anterior segment dysgenesis, vision problems, brain malformations, congenital defects of the cardiac septa and valves, mild to moderate hearing impairment, eye movement abnormalities, hypotonia, mild developmental delay and dysmorphic features. These characteristics were observed in all subgroups where FOXC1 was included in the deletion, confirming a dominant role for this gene. Additional characteristics were seen in individuals with terminal deletions exceeding 4.02 Mb, namely complex heart defects, corpus callosum abnormalities, kidney abnormalities and orofacial clefting. Some of these additional features may be related to the loss of other genes in the terminal 6p region, such as RREB1 for the cardiac phenotypes and TUBB2A and TUBB2B for the cerebral phenotypes. In the newly identified patients, we observed previously unreported features including gastrointestinal problems, neurological abnormalities, balance problems and sleep disturbances.ConclusionsWe present an overview of the phenotypic characteristics observed in terminal and subterminal 6p deletions. This reveals a common phenotype that can be highly attributable to haploinsufficiency of FOXC1, with a possible additional effect of other genes in the 6p25 region. We also delineate the developmental abilities of affected individuals and report on previously unrecognised features, showing the added benefit of collecting information directly from parents. Based on our overview, we provide recommendations for clinical surveillance to support clinicians, patients and families.

A Preliminary Examination of the use of Simultaneous Prompting to Teach Math Content to Students with Disabilities

This manuscript presents an examination of 15 experimental studies that investigated the use of the simultaneous prompting procedure to teach math content to students with disabilities, which were reported to be a mild or moderate intellectual disability, autism, or hearing impairment. A nearly equal number of elementary, middle, and high school participants were involved in the investigations, which were conducted in general and special education classrooms, participants’ homes, clinics, and university-based research centers. A single subject research design was used in each study, and both discrete and chained math skills were targeted. A visual analysis of the results, based on percentage of non-overlapping data, indicates variable support for the effectiveness of the procedure with all 35 of the participants. Likewise, in the studies that reported maintenance and generalization data, the results were variable. Altogether, this descriptive report serves as a preliminary examination to guide the work of teachers and researchers.

Effect of Age and Hearing Loss on Auditory Processing Skills.

Background: The present study aimed to evaluate the effect of age and hearing loss on auditory processing abilities. For this purpose, auditory processing abilities were compared among young and older adults with normal hearing sensitivity and older adults with and without hearing loss. Method: The study comprised 20 normal-hearing young adults (18-25 years), 20 older adults with normal hearing sensitivity (50-70 years), and 20 older adults with mild to moderate sensorineural hearing impairment (50-70 years). All 60 participants underwent gap detection(GDT), dichotic consonant-vowe(DCV)l, speech in noise(SPIN), duration pattern(DPT), and working memory (forward and backward span) tests in a sound treated test room. Results: Results showed that young normal-hearing adults performed significantly better than normal-hearing older adults in SPIN, GDT, DCV, working memory, and DPT. Futher, normal-hearing older individuals performed better than older individuals with hearing impairment on all the auditory processing tests except on the forward span test and DPT. Conclusion: Auditory processing abilities deteriorate with age and hearing loss also has a pronounced effect on most auditory processing abilities.

974: FEASIBILITY OF OTOACOUSTIC EMISSIONS TESTING TO IDENTIFY HEARING IMPAIRMENT IN THE ICU

Introduction: Hearing impairment is associated with a lack of functional recovery among older adults who have survived intensive care unit (ICU) admission and is a risk factor for delirium. Identifying hearing impairment in the ICU is challenging because many patients cannot participate in hearing assessments. Otoacoustic emissions testing (OAE) is a non-invasive, non-participatory tool that is used to screen newborns for hearing impairment by detecting intracochlear motion in response to auditory stimulation. Our objective was to assess the feasibility of OAE testing to screen for hearing impairment among adults in the ICU. Methods: A feasibility study was performed in the adult Medical Intensive Care Unit at Yale-New Haven Hospital. Data were gathered on demographics, delirium, ventilation status, sedation, illness severity, OAE measurements, and ambient noise. Percentage of test completion, the average time of test completion, and barriers or facilitators of testing were measured as outcomes. A patient passed their OAE testing if at least 2 of 6 frequencies were detected in at least one ear. A pass suggested that moderate or severe hearing impairment (i.e., that would require an amplifier or a hearing aid) was not present. Results: Of 31 patients approached (mean age 64.58+/-14.06 years), 23 (74.2%) underwent testing. Among all patients, 15 (48.4%) were mechanically ventilated, 8 (25.8%) were receiving sedative infusions, and 19 (61.3%) screened positive for delirium with the CAM-ICU. Eight (25.8%) patients were unable to be tested, most commonly due to elevated ambient noise (41.6%). Among the 18 patients with complete data, 6 patients screened positive for hearing impairment. The average time for OAE test completion per ear was 152.59+/-97.57 seconds. The average ambient noise level was 50.68+/-3.96 dB for all patients, and 52.41+/-2.08 dB among those who could not be tested in both ears. Conclusions: OAE testing is a feasible method for hearing impairment screening in the ICU. Identification of hearing impairment may facilitate improved communication through interventions such as amplifiers, audiology referrals, and accommodations. Future studies should evaluate whether identification and treatment of hearing impairment in the ICU may reduce delirium and improve post-ICU recovery.

Analysis of Serum Inflammatory Markers in Infants Under 6 Months of Age with Non-Syndromic Moderate and Severe Hearing Loss Associated with GJB2 Gene Mutations.

BACKGROUND The GJB2 gene is reported to be the main hereditary factor responsible for non-syndromic hearing impairment in infants. Several kinds of hearing loss have been linked to elevated inflammatory markers. This study aimed to evaluate serum levels of IL-2, IL-4, IL-6, IL-10, IL-17, alpha-TNF, and γ-IFN and the severity of hearing loss. MATERIAL AND METHODS Ninety newborns were divided into 3 groups: severe hearing impairment (31 infants), moderate hearing impairment (30 infants), and normal hearing (29 infants). Hearing screening was performed using otoacoustic emissions test. Mutations of the GJB2 gene were detected with Sanger sequencing. The patients had DNFB1 mutation. Seven blood inflammatory markers were tested using Cytometric Bead Array. We performed the t test to examine differences in expression of 7 inflammatory markers between sexes in the groups. The correlation between indicators within groups was studied using the Pearson correlation test. Correlation of different indicators among groups was studied using the Spearman correlation test. RESULTS When compared among the 3 groups (severe, moderate hearing impairment, and normal hearing group), we found that IL-10 had a positive correlation with the severity of GJB2-associated hearing loss, which was statistically significant (P<0.05). CONCLUSIONS This research aimed to assess the relationship of 7 serum inflammatory markers with GJB2-associated hearing loss in infants. Inflammatory marker IL-10 had a positive correlation with the severity of GJB2-associated infant hearing loss, and it might have the potential to become a future therapeutic target.

Prevalence of hearing loss in schoolchildren: populational study and global estimates

Introduction. Hearing impairments in schoolchildren affects learning and communication. Reliable data on hearing loss prevalence in this group are necessary for planning the audiological care.Aim. To compare data from a population-based study of the hearing loss prevalence in schoolchildren and estimates from the Global Burden of Disease (GBD) study in the Republic of Yemen.Materials and methods. Data extraction was performed from the GBD database on the prevalence of bilateral hearing loss &gt;20 dB among children aged 5–9 years in the Republic of Yemen. A two-stage study of hearing loss prevalence among primary school students aged 6–9 years in Sana’a, Republic of Yemen, in a sample of 2200 children using screening audiometry at 20 dB at 0.5, 1, 2, and 4 kHz is school settings, followed by tonal threshold audiometry and tympanometry in a specialized clinic. Hearing loss was assessed as unilateral or bilateral when hearing thresholds were higher than 25 dB.Results. According to GBD estimates, bilateral hearing impairment in this population is 2.2%, including 1.4% of mild degree. According to the results of a Yemenian population study, mild and moderate hearing impairment was detected in 10.6%, of which 6.8% – unilateral, 3.8% – bilateral hearing loss. There were no cases of severe or profound hearing loss. The prevalence of otitis media with effusion was 6%, chronic suppurative otitis media – 1.8%, sensorineural hearing loss – 1.6%.Conclusion. Bilateral hearing impairments occur in 2.2–3.8% of primary school students, with unilateral impairments the prevalence is up to 10.6%. Of these, at least 65% have conductive hearing loss due to pathology of the middle ear. The data obtained will improve the efficiency of ear and hearing care for schoolchildren.

Risk factors for hearing impairment in infants and toddlers in the Gaza governorates: a case-control study

BackgroundThe risk factors for congenital or early-onset hearing loss vary across countries. This study aimed to explore the risk factors for hearing impairment in children younger than 3 years in Gaza governorates to inform preventive strategies. MethodsThis case-control study started in February, 2017, and ended in July, 2018. Ethical approval was obtained from the Helsinki Committee in Palestine, a national committee established in 1986 that reviews all proposals for heath research, and whose approval is a requirement before any health research in Palestine. Data were collected through face-to-face household interviews. Cases were defined as children diagnosed with moderate, severe, or profound sensorineural hearing impairment within the first 3 years of life (n=169), whereas controls were children who had been screened and found to have unimpaired hearing (n=169). Cases and controls were randomly selected through a systematic sampling approach from the screening lists of the Atfaluna Society for Deaf Children, a registered Palestinian non-governmental organisation that has been working in the field of deaf education and allied services since 1992 and that regularly leads hearing screening campaign for neonates and children younger than 5 years in primary health centres over all Gaza governorates. SPSS version 21 was used for data entry and logistic regression analysis with the chi-square test, odds ratio, and t test. FindingsMaternal unemployment was more prevalent in cases than in controls (p=0·025). Having a family history and consanguinity were more prevalent in cases than in controls (p=0·001 for both). Taking folic acid during the preconception period (p=0·007) and iron supplements during pregnancy (p=0·002) were associated with a lower prevalence of hearing loss. The proportion of mothers of cases who were registered as having received antenatal care during the first trimester was lower for cases than for controls (odds ratio 0·341 [95% CI 0·181–0·640]; p=0·002). Prematurity (p=0·006), low birthweight (p<0·0001), and admission to a neonatal intensive care unit (p=0·002) were strongly associated with hearing impairments. The study also showed that children with recurrent otitis media were at a greater risk of hearing impairment (p<0·0001), as were children who had been exposed to sporadic loud noises, such as nearby bombing, or continuous loud noise at home, such as from nearby factories. (p=0·01). InterpretationThe study concluded that hearing impairment among children younger than 3 years is associated with a wide array of socioeconomic, health, and environmental vulnerabilities. A health promotion programme should be established to address consanguinity, prematurity prevention, and timely and appropriate preconception and antenatal care, alongside universal screening for all children, particularly those at risk as identified in this study. FundingNone.

Association of Hearing Impairment and 24-Hour Total Movement Activity in a Representative Sample of US Adults

Lower physical activity (PA) levels have been proposed as a mechanism to explain the association between hearing loss and various adverse outcomes of aging. But whether hearing loss is associated with lower PA is poorly understood. To evaluate whether hearing loss is associated with lower levels of PA. This cross-sectional analysis was conducted in a representative sample of US adults (aged 30-69 years) who participated in the 2011-2012 cycle of the National Health and Nutrition Examination Survey (NHANES). Data analysis was conducted from July to September 2021. Hearing was assessed, in dB, by pure-tone audiometry and summarized as the better hearing ear's pure-tone average (BPTA) of 4 speech frequencies (500, 1000, 2000, and 4000 kHz); a higher BTPA indicates worse hearing. Hearing was also categorized into normal (BPTA ≤25 dB), mild hearing impairment (>25 to 40 dB), and moderate or greater hearing impairment (>40 dB). Total 24-hour movement activity was measured using wrist accelerometers worn at all times for 7 consecutive days and summarized in monitor-independent movement summary units (MIMS) units (higher MIMS units indicate more movement). The association between BPTA and MIMS units was evaluated using a multivariable-adjusted (demographic characteristics and chronic conditions) piecewise spline regression (knot at 15 dB). Mean differences in MIMS units across hearing categories compared with normal hearing were estimated. The study included 2490 participants (mean [SE] age, 48.9 [0.3] years; 900 [weighted percentage, 69.3%] White individuals, 1248 [weighted percentage, 50.8%] female participants). Before the knot at 15 dB, we found that each 10-dB higher BPTA was associated with 860.4 (95% CI, 444.8-1276.1) higher MIMS units. Conversely, after the knot, each 10-dB higher BPTA was associated with 458.6 (95% CI, 27.7-889.4) lower MIMS units. The difference in MIMS units across hearing categories was not statistically significant. In this cross-sectional study, in the range of hearing sensitivity of BPTA of 15 dB or greater, worse hearing was associated with lower MIMS units. Lower PA may be a mechanism contributing to the association between hearing impairment and adverse health.