Sensorineural Research Articles

Effects of Frequency-Composition Algorithm and Phoneme-Based Auditory Training in Older Hearing Aid Users.

Frequency-lowering (FL) algorithms improve the audibility of high-frequency sounds by shifting inaudible high-frequency components to audible low-frequency regions. However, the FL algorithm has yielded mixed findings. This study involved two experiments. The first experiment investigated whether objective and subjective auditory outcomes would be enhanced by activating frequency composition (Fcomp), which is a type of FL technique. The second experiment determined whether auditory training with Fcomp activation would provide perceptual benefits to older hearing aid users. Twelve older hearing aid users with moderate to profound high-frequency sensorineural hearing loss participated in this study. In Experiment I, all participants received a 4-week adaptation period to Fcomp before the measurement, and then the influence of Fcomp was evaluated. In Experiment II, among the 12 participants in Experiment I, five received 8-week phoneme-based auditory training while activating Fcomp, whereas the remaining seven did not receive training but continued to use Fcomp as non-trained controls. In Experiment I, 4-week passive accli-matization period to Fcomp did not improve speech-in-quiet recognition or self-perceived sound quality. In Experiment II, active pho-neme-based training enhanced recognition of consonants and words as well as better speech-quality ratings for trained participants. The consecutive use of Fcomp did not lead to any differences for non-trained participants. Overall, phoneme-based auditory training would allow older hearing aid users to relearn frequency-lowered speech sounds and reduce phonetic confusion. However, the analytical training approach did not lead to transfer to sentence recognition or overall satisfaction with the hearing aids.

Relationship between hearing impairment and dementia and cognitive function: a Mendelian randomization study

BackgroundThere is a substantial body of observational research indicating an association between hearing impairment and dementia, yet the causal relationship and underlying mechanisms remain uncertain. This study aims to investigate the causal relationship between hearing impairment and its subtypes with dementia and cognitive function using two-sample Mendelian randomization (MR) analysis.MethodsWe performed two-sample MR analysis to examine the causal effects of hearing impairment and its subtypes, including conductive and sensorineural hearing loss (CSHL), conductive hearing loss (CHL), sensorineural hearing loss (SHL), and sudden sensorineural hearing loss (SIHL), on six dementia phenotypes (overall dementia, Alzheimer’s disease [AD], Lewy body dementia [DLB], frontotemporal dementia [FTD], Parkinson’s disease dementia, and vascular dementia) and four cognitive functions. Additionally, multivariable MR (MVMR) analysis was employed to investigate potential mediating mechanisms.ResultsGenetically determined CSHL was associated with an elevated risk of DLB (odds ratio [OR] 1.69; 95% CI 1.08 to 2.63; P = 0.021) and FTD (OR 1.66; 1.04 to 2.67; P = 0.035), but not AD (P = 0.958). Genetic predisposition to CHL was found to link with increased risks of AD (OR 1.07; 1.01 to 1.14; P = 0.031). Genetically determined SHL was causally associated with an elevated risk of semantic dementia (OR 3.81; 1.09 to 13.37; P = 0.037). Genetically predicted CHL and SIHL were both causally associated with lower general cognitive performance (β -0.015 and − 0.043; P = 0.007 and 0.013) and fluid intelligence score (β -0.045 and − 0.095; P = 0.037 and 0.040). In MVMR analysis, the causal relationship between hearing impairment and dementia was mediated by loneliness, depressed mood, and brain cortical volume, particularly the medial temporal lobe, but not by aging or ischemic stroke.ConclusionOverall, the study provides evidence supporting a causal relationship between hearing impairment and increased risks of different types of dementia (including AD, FTD, and DLB), as well as poorer general cognitive function. These findings underscore the importance of addressing hearing impairment as a modifiable risk factor for dementia.

Objective audiometric evaluation of individuals with type 2 diabetes mellitus: A cross-sectional survey in South West Nigeria

Aims: Diabetes mellitus affects more than 450 million people around the world. The relationship between diabetes and sensorineural hearing loss has been an area of interest. The aim of this study is to evaluate hearing loss, among type 2 diabetes diagnosed in the endocrinology unit of a private tertiary health facility in South West Nigeria. Methods: This was a hospital-based cross-sectional study carried out among type 2 diabetic patients and normal healthy non-diabetic individuals as control. A total of 120 participants were recruited for the test and control. Otoacoustic emission and auditory steady-state response audiometry were carried out on all the participants. Result: The total number of study participants was 120 (60 for test and control) with an age range of 30–79 years for the test and 20–80 years for the control. Otoacoustic emission showed passes for both ears, 61.7% pass in both ears for the test group and 76.7% pass in both ears for the control group. 21.7% of the test and control were referred for both ears. 16.6% of the test group was referred in one ear compared to 1.7% in the control group. Auditory steady-state response (ASSR) audiometry showed mild to profound hearing loss in all test groups while in the control group 20% had normal hearing. Moderate to moderately severe hearing loss in at least one ear was 38.4% in the test ear compared to 16.7% in the control group. Conclusion: Hearing loss is an established complication of type 2 diabetes. Therefore, it is necessary to incorporate regular hearing screening for patients with diabetes for early detection, counseling, and rehabilitation for those with disabling hearing loss.

Phenotyping vestibulocochlear manifestations in Susac syndrome: a cohort study.

To characterize vestibulocochlear involvement in patients with Susac syndrome (SuS), a rare immune-mediated endotheliopathy of cerebral, retinal and inner ear microvasculature causing a triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. The electronic patient files of 21 patients with SuS are reviewed for data on demography, clinical presentation, disease course and audiovestibular testing. All 21 patients experienced some form of audiovestibular complaints during the disease course, with vertigo and instability being most frequently reported, followed by hearing loss, tinnitus and aural fullness. These audiovestibular symptoms did not always coincide. Fifteen patients had objectified predominant low- to midfrequency sensorineural hearing loss and 8 out of 18 patients showed abnormalities on vestibular testing, most frequently vestibular evoked myogenic potential-abnormalities, indicating otolith dysfunction. Treatment protocols consisted of uniformly extensive immunosuppressive therapy and hearing loss remained mostly mild. Audiovestibular involvement is very common in patients with SuS. Characteristic findings include a "reverse-slope" configuration on audiological testing and otolith dysfunction on vestibular testing. Aggressive immunosuppression may prevent severe audiovestibular dysfunction. Symptoms as aural fullness and otolith dysfunction may indicate an underlying hydrops. Further investigations are necessary to elucidate the histopathological mechanisms underlying these preferentially involved cochleovestibular areas. Early recognition and treatment of SuS are important to stabilize or decrease disease activity and might also have beneficial effects on inner ear outcome. THE SUBMITTED MANUSCRIPT REPORTS DATA DERIVED FROM CLINICAL OBSERVATIONS IN HUMANS: Consent for the research was provided by the Ethics Committee of Ghent University hospital (application number 2019/1443, registration date 31/12/2021, principal investigator Guy Laureys).

The Role of Pericytes in Inner Ear Disorders: A Comprehensive Review.

Inner ear disorders, including sensorineural hearing loss, Meniere's disease, and vestibular neuritis, are prevalent conditions that significantly impact the quality of life. Despite their high incidence, the underlying pathophysiology of these disorders remains elusive, and current treatment options are often inadequate. Emerging evidence suggests that pericytes, a type of vascular mural cell specialized to maintain the integrity and function of the microvasculature, may play a crucial role in the development and progression of inner ear disorders. The pericytes are present in the microvasculature of both the cochlea and the vestibular system, where they regulate blood flow, maintain the blood-labyrinth barrier, facilitate angiogenesis, and provide trophic support to neurons. Understanding their role in inner ear disorders may provide valuable insights into the pathophysiology of these conditions and lead to the development of novel diagnostic and therapeutic strategies, improving the standard of living. This comprehensive review aims to provide a detailed overview of the role of pericytes in inner ear disorders, highlighting the anatomy and physiology in the microvasculature, and analyzing the mechanisms that contribute to the development of the disorders. Furthermore, we explore the potential pericyte-targeted therapies, including antioxidant, anti-inflammatory, and angiogenic approaches, as well as gene therapy strategies.

Congenital Cytomegalovirus Severity Definitions and Treatment Decisions around the World: A Systematic Scoping Review of the Literature.

Congenital cytomegalovirus (cCMV) is the most common cause of congenital infection and the leading cause of non-genetic sensorineural hearing loss in childhood. While treatment trials have been conducted in symptomatic children, defining asymptomatic infection can be complex. We performed a scoping review to understand how infection severity is defined and treated globally, as well as the various indications for initiating treatment. We conducted an electronic search of MEDLINE, EMBASE, Scopus, and the Cochrane Library, using combinations of the following terms: "newborn", "baby", "child", "ganciclovir", "valganciclovir", and "cytomegalovirus" or "CMV". We included eligible prospective and retrospective studies, case series, and randomized clinical trials (RCTs) published up to May 2024. A total of 26 studies were included, of which only 5 were RCTs. There was significant heterogeneity between studies. The most commonly considered criteria for symptomatic infection were microcephaly (23/24 studies), abnormal neuroimaging (22/24 studies), chorioretinitis/ocular impairment (21/24 studies), and hearing impairment (20/24 studies). Two studies also included asymptomatic newborns in their treatment protocols. Outcome measures varied widely, focusing either on different hearing assessments or neurocognitive issues. Our literature analysis revealed significant variability and heterogeneity in the definition of symptomatic cCMV infection and, consequently, in treatment approaches. A consensus on core outcomes and well-conducted RCTs are needed to establish treatment protocols for specific groups of newborns with varying manifestations of cCMV.

8242 A Case of Ayme Gripp Syndrome

Abstract Disclosure: P. Jaisinghani: Consulting Fee; Self; Novo Nordisk. J. Pappas: None. We present a 35 year old female with cataracts, generalized seizures and oligodontia, referred to us for hair loss and elevated TSH. For over 10 years, patient had a diffuse hair loss which progressed to a focal patch of hair loss in the setting of prior use of keppra and seborrheic dermatitis. Patient has regular menses, and no symptoms of hyper/hypothyroidism or family history of thyroid disease. Patient established care with dermatology and scalp biopsy noted early androgenetic alopecia. Physical exam was notable for short stature, height 4'11, 98 lbs, BMI 19.8 kg/m², progeric appearance, facial hyperpigmented spot, hair thinning, palpable thyroid R>L. Laboratory evaluation included TSH 6.150 (0.450 - 4.500 uIU/mL), DHEAS 263 (84.8 - 378.0 ug/dL), total testosterone 15 (10 - 55 ng/dL), FSH 6.6 (Follicular phase 3.5 - 12.5 mIU/mL), LH 7.6 (Follicular phase 2.4 - 12.5 mIU/mL), estradiol 83 (Follicular phase 12.5 - 166.0 pg/mL). Thyroid US demonstrated smaller than average size left lobe measuring 2.93 cm x 0.84 cm x 1.36cm vs right lobe measured 4.34 cm x 0.90 cm x 1.62. Labs noted TSH 5.690 (0.450 - 4.500 uIU/mL), establishing subclinical hypothyroidism and negative thyroid antibodies. Clinical genetics evaluation included trio exome sequencing which revealed a de novo pathogenic variant p.(Ser54Leu) (TCG>TTG): c.161 C>T in exon 1 of the MAF gene (NM_005360.4) which establishes the diagnosis of a Ayme- Gripp syndrome (AGS). AGS is characterized by a triad of cataract, sensorineural hearing loss, and characteristic facial features in combination with neurodevelopmental abnormalities. 21 affected individuals from 19 families have been reported with AGS. Clinical features in our case characteristic of AGS included short stature, brachycephaly, midface hypoplasia, long philtrum and premature hair loss. Hypothyroidism was identified in childhood in 3/15 identified individuals who responded to thyroid hormone replacement therapy. For this patient, we will monitor thyroid function tests every 3-6 months and monitor for symptoms of hypothyroidism. Patient has been referred to cardiology and nephrology, and will continue to follow with genetics and dermatology. Our case further establishes the association of hypothyroidism with AGS. Our case demonstrates the value of expanding the differential of endocrinopathies to rare genetic conditions which are currently identifiable by clinically available genomic tests. Presentation: 6/1/2024

Unilateral Sensorineural Hearing Loss in Congenital Cytomegalovirus Retrospective Observational Study.

The leading nonhereditary cause of childhood sensorineural hearing loss has been attributed to congenital cytomegalovirus (cCMV). Sensorineural hearing loss can be unilateral (UHL) or bilateral (BHL), and may be progressive. Our objective was to describe the characteristics, clinical nature and follow-up of ears in cCMV-associated UHL. This 16-year retrospective study was performed at Schneider's Medical Center, Israel. Data were collected from all cCMV infants with UHL at birth who were treated with antiviral treatment initiated within the first 4 weeks of life and had a follow-up period of at least 1 year. We enrolled 67 infants diagnosed with UHL at birth: 17 (25%) with mild hearing loss, 22 (33%) with moderate hearing loss and 28 (42%) with severe hearing loss. At the last follow-up visit, 7 (41%) ears in the mild hearing loss group improved to normal hearing, 8 (47%) ears exhibited no change and 2 (12%) ears deteriorated. In the moderate hearing loss group, 9 (41%) ears improved, 7 (32%) remained static and 6 (27%) deteriorated to the severe hearing loss group. One (4%) ear in the severe hearing loss group showed improvement. Of the 67 ears with normal hearing at birth, 4 (6%) ears deteriorated. This study assessed and elucidated the characteristics, clinical nature and long-term follow-up of both the affected and unaffected ears diagnosed with UHL due to cCMV. These data are crucial when medical and/or surgical interventions are considered.

Outcome of Children with Congenital Cytomegalovirus Infection: A Retrospective Observational Study

Abstract Objective Congenital cytomegalovirus (CMV) infection is a common intrauterine infection and the leading cause of nonhereditary sensorineural hearing loss. This study aims to assess the long-term outcome of the infection and to identify infants at risk of developing long-term sequelae. Methods This retrospective single-center observational study includes infants born between 2003 and 2019 with confirmed congenital CMV (based on current criteria). Brain imaging (ultrasound and magnetic resonance imaging [MRI]), clinical monitoring of neurosensory development, and auditory brainstem evoked responses were performed, as well as long-term neurodevelopmental follow-up to assess sequelae. Results A total of 66 infants with congenital CMV were included in the study. Median gestational age at birth was 38.6 weeks (interquartile range: 36.9–40.1). Clinical findings included intrauterine growth restriction (39%), microcephaly (29%), thrombocytopenia (17%), and jaundice (11%). Brain abnormalities were observed on ultrasound (30%) and MRI (42%). Neurodevelopmental scores were abnormal in 21 subjects (43%) and associate with the vacuolization of anterior temporal lobe and ventricular septations on MRI (both p = 0.05). Fourteen patients (21%) had sensorineural hearing loss, which was more common in patients with abnormal cerebral images at birth, determined by ultrasound (p = 0.06). Microcephaly (p = 0.05) and abnormal MRI (p = 0.03) at birth were associated with poor long-term outcomes. Conclusion Early detection of congenital CMV infection is important to prevent long-term complications in affected infants. Understanding the predictors of poor outcomes may help improving management and treatment strategies for this condition.

Prenatal and postnatal antiviral therapies for the prevention and treatment of congenital cytomegalovirus infections.

Congenital cytomegalovirus infection (cCMV) is the leading infectious cause of sensorineural hearing loss and lifelong neurodevelopmental disabilities. Studies suggest antiviral therapy can prevent fetal infection after maternal primary infection, as well as halt the progression of hearing loss and neurodevelopmental disabilities in newborns with symptomatic cCMV. With growing worldwide momentum on early detection and diagnosis of cCMV, this review describes the exciting recent advances in antiviral therapies in CMV infected pregnant mothers and babies, as well as emerging evidence on anti-CMV vaccines. New opportunities for prenatal and neonatal interventions have driven a rising interest in screening and identification of asymptomatic CMV infection. Routine screening of pregnant women to identify primary infection in first trimester is now advocated in Western Europe but has yet to be examined from a public health perspective in other regions. Evidence is emerging for maternal valaciclovir therapy to prevent fetal infection after a maternal primary CMV infection in the first trimester of pregnancy. For those infants who are born with symptomatic cCMV, a 6-month course of valganciclovir, started within the first 4 weeks of life, and possibly up to 13 weeks of life, is the current recommended therapy. However, there is unclear evidence for the benefit of treatment for asymptomatic cCMV and cCMV with isolated hearing loss. Research to identify more effective antivirals and an effective CMV vaccine continues. More research is needed to determine the region-specific applicability of the new European recommendations for routine CMV screening in pregnancy. Areas of uncertainty in postnatal management include timing of initiation, duration of treatment and identifying pediatric subgroups that benefit from modification of the standard treatment recommendations.

Evaluation of angiotensin converting enzyme insertion/deletion, alpha adducin (ADD1) G460W, and IL-10 gene polymorphisms, and determination of prognostic effects in idiopathic sudden sensorineural hearing loss

ObjectiveThe aim of this study was to examine angiotensin converting enzyme (ACE) insertion/deletion, alpha adducin, and interleukin-10 (IL-10) gene polymorphisms (GPs) in terms of both idiopathic sudden sensorineural hearing loss (ISSNHL) risk and their potential prognostic effects. MethodsThe study group consisted of 70 patients and the control group consisted of 50 patients. Venous blood samples were analyzed for relevant GPs via kompetitive allele-specific polymerase chain reaction. Age, sex, affected side, tinnitus, and vertiginous symptom status, number of days between symptom onset and hospital admission, pure tone audiometry results at admission and after treatment were included in the study. Data were compared statistically. ResultsThe D allele of ACE insertion/deletion GP was significantly more frequent in patients with ISSNHL than in the control group (p = 0.032). II genotype was associated with a reduced risk of ISSNHL (p = 0.036). The amount of hearing loss was significantly higher in patients with the TT genotype (p = 0.027) and T allele of the IL-10 GP (p = 0.035) than in the patients without this allele. Severe hearing loss was a poor prognostic factor (p = 0.008). ConclusionsThe D allele of ACE insertion/deletion GP may be involved in the ISSNHL etiology. Due to the association of this allele with occlusive vascular pathologies, ischemia is believed to be a common pathway in the etiopathogenesis of ISSNHL.

Spectral weighting functions for localization of complex sound. III. The effect of sensorineural hearing lossa).

Spectral weighting functions for sound localization were measured in participants with bilateral mild sloping to moderately severe, high-frequency sensorineural hearing loss (SNHL) and compared to normal hearing (NH) participants with and without simulated SNHL. Each participant group localized three types of complex tones, comprised of seven frequency components spatially jittered and presented from the horizontal frontal field. A threshold-elevating noise masker was implemented in the free field to simulate SNHL for participants with NH. On average, participants with SNHL and NH (in quiet and simulated SNHL) placed the greatest perceptual weight on components within the interaural time difference "dominance region," found previously to peak around 800 Hz [Folkerts and Stecker, J. Acoust. Soc. Am. 151, 3409-3425 (2022)]. In addition to the peak at 800 Hz, both participant groups (including NH participants in quiet) placed near equal weight on 400 Hz, resulting in a broadened "peak" in the dominance region, most likely due to the reduction of audibility to higher frequency components. However, individual weighting strategies were more variable across participants with SNHL than participants with NH. Localization performance was reduced for participants with SNHL but not for NH participants with simulated hearing loss when compared to NH participants in quiet.

Atypical Mumps; are We Heading Towards an Outbreak?

Mumps, caused by the mumps virus, is a contagious disease primarily affecting children and young adults. While typically presenting with salivary gland swelling and systemic symptoms, mumps can lead to various complications including SNHL, orchitis/ oophoritis, aseptic meningitis. Recent observations suggest atypical features in mumps cases, raising concerns of a potential outbreak in India. To discuss the etiopathogenesis and clinical presentation in cases of atypical mumps with increasing number of cases, a prospective multicentric study was conducted across five major centers - SMS Medical College Jaipur, RDBP Jaipuria Hospital, Jaipur, Shri Ashwini Saxena ENT Hospital Rewari, AIIMS Bhubaneswar and SP Medical College Bikaner, in India to evaluate patients with acute salivary gland swellings. Clinical and laboratory data were collected, including demographics, presenting symptoms, history of vaccination, imaging findings, and treatment outcomes. Patients were followed for four weeks post-treatment to monitor for delayed complications. Among 53 patients, a bimodal age distribution was observed, with peaks in early adolescents and middle-aged individuals. Vaccination status was recorded based on recall. Fever and salivary gland swelling were predominant symptoms, with a significant proportion experiencing submandibular gland involvement. Elevated serum amylase and CRP levels correlated with disease severity and prolonged symptomatic resolution. Notably, cases of sensorineural hearing loss (SNHL) and airway complications emerged as significant concerns. The study highlights a shift in mumps demographics, with higher age groups affected and increased incidence of complications like SNHL and airway compromise. International trends also suggest periodic outbreaks and evolving clinical manifestations post-COVID-19 pandemic. Factors contributing to mumps resurgence include lack of vaccination or vaccine efficacy, population immunity, and seasonal variations. India appears to be facing a potential mumps outbreak, characterized by atypical features and increased risk of complications like SNHL and airway compromise. Serum amylase and CRP serve as valuable markers for disease severity. Early recognition and management of complications are crucial, emphasizing the importance of mumps immunization to mitigate the impact of the disease. ENT specialists should remain vigilant for emerging complications, particularly SNHL, advocating for comprehensive immunization strategies.

Analysis of Characteristics and Prognostic Factors of Full-Frequency Idiopathic Sudden Sensorineural Hearing Loss with Hyperlipidemia.

To explore the relationship between hyperlipidemia and full-frequency idiopathic sudden sensorineural hearing loss (FFHL). A total of 145 FFHL patients admitted from September 2021 to May 2024 were analyzed. Eighty-five patients with hyperlipidemia and 60 patients with normal serum lipids statistically analyze the patient's gender, age, onset time, accompanying symptoms, and serum lipids. Measure the pure tone hearing threshold before and after treatment, and calculate the average increase in hearing threshold. Compare the correlation between various factors and prognosis. The hyperlipidemia group had a higher proportion of males (P=0.043) and vertigo (P=0.044) compared to the control group, while other information between the two groups lacked statistical significance. Effective patients (n=44) in the hyperlipidemia group showed significant differences in vertigo (P=0.020), age (P=0.032), and onset (P=0.030) compared to ineffective patients (n=41). Analysis showed that these were independent correlated factors affecting prognosis. Other indicators, including serum lipids, have no significance in predicting the prognosis of patients with hyperlipidemia and complete frequency decline in hearing loss. Patients with hyperlipidemia have higher rates of males and vertigo. The age, onset, and vertigo of patients with hyperlipidemia are related to prognosis, while various lipid indicators are not related to the prognosis of sudden hearing loss.

Genetic testing for pediatric sensorineural hearing loss in the era of gene therapy.

To summarize indications, methods, and diagnostic yields for genetic testing for pediatric hearing loss. Genetic testing has become a cornerstone of clinical care for children with sensorineural hearing loss. Recent studies have shown the efficacy of gene panels and exome sequencing for any child with sensorineural hearing loss. Recent findings have underscored the importance of a diagnosis in clinical care. Clinical trials for gene therapy for hearing loss have begun. Genetic testing has become critical for personalized care for children with hearing loss. Recent studies have shown a 43% overall diagnostic yield for genetic testing for pediatric hearing loss, though the diagnostic yield may range from 10 to 60% depending on clinical features. Syndromic diagnoses comprise 25% of positive genetic tests for pediatric sensorineural hearing loss. While diagnostic yield is lower for children with unilateral or asymmetric sensorineural hearing loss, the likelihood of syndromic hearing loss finding is higher. An early and accurate genetic diagnosis is required for participating in clinical trials for gene therapy for hearing loss.

Perilymphatic enhancement and endolymphatic hydrops: MRI findings and clinical associations.

In this study, we aimed to summarize magnetic resonance imaging (MRI) findings of perilymphatic enhancement (PE) and endolymphatic hydrops (EH) of the inner ear, which are associated with vestibular and cochlear symptoms. We analyzed data on ears with definite Meniere's disease (MD), sensorineural hearing loss (SNHL), vertigo, and listening difficulties (LiD) from 508 ears of 254 patients who underwent contrast-enhanced 3-Tesla MRI between April 2021 and March 2023. We evaluated the degree of endolymphatic hydrops (EH), signal intensity ratios (SIRs) between the basal turns of the cochlea and cerebellum, and hearing levels for all ears. Ears with definite MD were also assessed for changes in vestibular and cochlear symptoms within 6 months. Ears with definite MD exhibited significantly higher percentages of EH in both the vestibule and cochlea compared with ears with other diseases. Furthermore, ears with MD or sensorineural hearing loss (SNHL) had significantly higher SIRs of PE compared with ears with other diseases or asymptomatic ears. Among patients with definite MD, those experiencing hearing fluctuations or vertigo attacks within the last 6 months had significantly higher SIRs of PE compared with those who did not experience any symptoms. Significant EH in the vestibule and cochlea was a major finding for the imaging diagnosis of definite MD. A high SIR of PE was a good indicator for assessing MD activity, reflecting vestibular and cochlear symptoms and fluctuations.Level of Evidence: 4.

Evaluation of Hearing Loss: Understanding Audiologic Testing to Refine Image Interpretation.

The standard of reference for diagnosing and characterizing hearing loss is audiologic testing. The results of audiologic testing inform the imaging algorithm and the differential diagnosis for the underlying cause. Pure-tone audiometry tests the ability to hear tones across different frequencies, and the results are displayed as an audiogram. Tympanometry measures tympanic membrane compliance as a function of pressure to generate a tympanogram. Acoustic reflex testing helps differentiate third window lesions from other causes of conductive hearing loss. Clinical and audiologic assessment of sensorineural hearing loss helps in differentiating cochlear from retrocochlear causes. Symmetrical sensorineural hearing loss is typical of cochlear disease. Asymmetry increases the likelihood of a retrocochlear lesion, the most common of which among adults is vestibular schwannoma. Unlike patients with sensorineural hearing loss, who commonly have normal imaging studies, patients with conductive hearing loss are expected to have abnormal temporal bone CT studies. By incorporating the results of audiologic testing into their evaluation, radiologists can perform a more informed and more intentional search for the structural cause of hearing loss. The authors describe several audiogram configurations that suggest specific underlying mechanisms of conductive hearing loss. By providing a practical and accessible summary of the basics of audiologic testing, the authors empower the radiologist to leverage relevant clinical information and audiologic test results to interpret temporal bone imaging more confidently and more accurately, particularly temporal bone CT in the setting of conductive hearing loss. ©RSNA, 2024.

Hearing and balance functions in patients with systemic lupus erythematosus

Background Systemic lupus erythematosus (SLE) is a chronic, inflammatory, autoimmune disease, characterized by multiple organ involvement, which is seen more often in young females. Objectives To evaluate the hearing and balance functions in SLE patients. Materials and methods Twenty-four SLE patients, 24 healthy controls underwent pure tone audiometry (0.125–16 kHz), VNG, vestibular evoked myogenic potential (VEMP), and video head impulse test (vHIT). Results A statistically significant difference was determined between the control group and the SLE group at 0.125, 0.25, 0.5, 1.0, 6.0, 8.0, 10, 12, 14, and 16 kHz frequencies. Sensorineural hearing loss was observed in 37.5% of the SLE group. The pursuit test was pathological at the rate of 25% in the SLE group. No response was obtained in six SLE patients in the oVEMP test and four SLE patients in the cVEMP test. The mean posterior SCC VOR gain values were 0.64 ± 0.2 in the SLE group. A significant difference was determined between the groups with respect to posterior SCC VOR gains. Conclusions and significance Our findings show that SLE disease may negatively affect the hearing system and the vestibular system as well. The current study is the first study to comprehensively evaluate SLE patients with VNG, VEMP, and vHIT tests.

Radiotherapy-associated Sensorineural Hearing Loss in Pediatric Oncology Patients.

During the radiotherapeutic treatment of pediatric oncology patients, they would be at a latent risk of developing ionizing radiation-induced ototoxicity when the cochlea or auditory nerve is located within the radiation field. Sensorineural hearing loss (SNHL) is an irreversible late complication of radiotherapy, and its incidence depends on various factors such as the patient's hearing sensitivity, total radiation dose to the cochlea, radiotherapy fractionation regimen, age and chemoradiation. Importantly, this complication exhibits serious challenges to adult survivors of childhood cancer, as it has been linked to impairments in academic achievement, psychosocial development, independent living skills, and employment in the survivor population. Therefore, early detection and proper management can alleviate academic, speech, language, social, and psychological morbidity arising from hearing deficits. In the present review, we have addressed issues such as underlying mechanisms of radiation-induced SNHL, audiometric findings of pediatric cancer patients treated with radiotherapy, and management and protection measures against radiation-induced ototoxicity.

A Novel De Novo Gain-of-Function CACNA1D Variant in Neurodevelopmental Disease With Congenital Tremor, Seizures, and Hypotonia.

De novo gain-of-function variants in the CACNA1D gene, encoding the L-type voltage-gated Ca2+ channel CaV1.3, cause a multifaceted syndrome. Patients show variable degrees of autism spectrum disorder, developmental delay, epilepsy, and other neurologic and endocrine abnormalities (primary aldosteronism and/or hyperinsulinemic hypoglycemia). We study here a novel variant [c.3506G>A, NM_000720.4, p.(G1169D)] in 2 children with the same CACNA1D mutation but different disease severity. The clinical data of the study patients were collected. After molecular analysis and cloning by site-directed mutagenesis, patch-clamp recordings of transfected tsA201 cells were conducted in whole-cell configuration. The functional effects of wild-type and mutated channels were analyzed. One child is a severely affected boy with a novel de novo CACNA1D variant with additional clinical symptoms including prenatal-onset tremor, congenital respiratory insufficiency requiring continuous positive airway pressure ventilation, and sensorineural deafness. Despite episodes of hypoglycemia, insulin levels were normal. Aldosterone:renin ratios as a screening parameter for primary aldosteronism were variable. In the second patient, putative mosaicism of the p.(G1169D) variant was associated with a less severe phenotype. Patch-clamp electrophysiology of the p.(G1169D) variant in a heterologous expression system revealed pronounced activity-enhancing gating changes, including a shift of channel activation and inactivation to more hyperpolarized potentials, as well as impaired channel inactivation and deactivation. Despite retained sensitivity to the Ca2+ channel blocker isradipine in vitro, no beneficial effects of isradipine or nifedipine treatment were observed in the index case. Through this report, we expand the knowledge about the disease presentation in patients with CACNA1D variants and show the novel variant's modulatory effects on CaV1.3 gating.