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AbstractBackgroundObjectivesMaterial and MethodsResultsConclusions and SignificanceThe effect of batroxobin on hearing recovery in patients with Sudden Sensorineural Hearing Loss (SSNHL) is still controversial, and acupuncture shows auxiliary benefits for SSNHLTo investigate the effectiveness of combining acupuncture with batroxobin therapy for patients with SSNHL.One hundred and fifty-six patients with SSNHL were retrospectively enrolled in this study, and categorized into the control group (only batroxobin treatment) and observation group (batroxobin and acupuncture treatment). Pure Tone Audiograms (PTA) threshold and clinical outcomes of hearing recovery were compared. Logistic regression analysis was used to evaluate the association between hearing recovery and potential risk factors.Compared to the control group, the observation group had a higher overall effective rate (p = 0.006) and improvement in PTA threshold (p = 0.007). Among SSNHL patients with high-frequency and flat-type hearing loss, observation group demonstrated superior hearing recovery post-treatment compared to the control group (p < 0.05). Additionally, hearing recovery in patient with SSNHL were associated with SSNHL types, disease duration, neutrophil count and acupuncture (p < 0.05).Combining batroxobin and acupuncture treatments enhences the improvement of hearing recovery in SSNHL patients compared to only batroxobin treatments, especially high-frequency and flat-type hearing loss. [ABSTRACT FROM AUTHOR]

To investigate retrospectively the frequency of usage of bimodal stimulation among cochlear implant (CI) users, as well its clinical benefit relative to unilateral use. All subjects had been monitored with the clinical Minimal Outcome Measurements test battery. 103 adults with bilateral postlingual profound sensorineural hearing loss and unilateral CI use were extracted from the local database. These were divided into two groups: those who only used a CI and those who used bimodal stimulation. The preoperative contralateral residual hearing in the bimodal group was significantly better than that of the CI-only group. In both groups, speech perception in quiet and in noise improved after CI, with no significant difference between postoperative unimodal conditions. For the bimodal group, an additional significant improvement was found for the bimodal condition compared to the unimodal. Given the observed auditory benefit of bimodal stimulation in comparison to unimodal stimulation and given the finding that degree of residual hearing is not correlated with bimodal benefits, it is recommended to encourage CI recipients to continue contralateral HA use after CI. As a result of expanding CI criteria worldwide, the population of bimodal users is expected to grow in the near future. [ABSTRACT FROM AUTHOR]

The aim of the research was to evaluate the feasibility of measuring the bone conduction hearing threshold using self-administered mobile audiometry. A single-centre, closed, cross-over trial was carried out on patients from the ENT Department. A mobile-based, self-administered, audiologist-assisted assessment of the bone conduction hearing threshold was carried out by means of the open-access, freeware app Hearing Test using two types of bone conduction headphones: professional B71 bone transducer and commercially available AfterShokz Openmove open-ear headphones. Seventy-seven ears. A test-retest examination revealed the lowest standard deviation for open-ear headphones at 3.33 dB (95% CI 2.92–3.79). When compared with pure tone audiometry, the intraclass correlations of 0.95 (95% CI 0.94–0.96) and 0.90 (95% CI 0.88–0.92) were obtained for the bone transducer and for the open-ear headphones, indicating excellent and good reliability, respectively. However, the regression slope of 0.67 was found for the air-bone gap when using open ear headphones, which was significantly different from 1 (p < 0.001). Open-ear headphones provide an alternative for estimating bone conduction once the air-bone gap has been adjusted by a factor of 1/0.6 7 ≅1.5. They demonstrate improved reproducibility over the bone transducer and are much easier to use with a mobile device. Trial Registration: Wroclaw Medical University, Science Support Centre, BW60/2020 [ABSTRACT FROM AUTHOR]

Consumer-grade insert earphones (IEs) can be utilised for audiometry, but their calibration values and threshold reliability may differ from the audiometric IE. This study measured the equivalent threshold sound pressure levels (ETSPLs), and the test-retest threshold variation when a consumer IE (Sennheiser CX100) was fitted with: (1) silicone stock ear tips that came along with the earphone, (2) replacement foam ear tips (KZ acoustics) and (3) silicone otoacoustic emission (OAE) probe ear tips. Study 1 determined ETSPL values in 25 normal-hearing subjects aged 18–25 years at seven test frequencies (500–8000 Hz). Study 2 assessed the intra- and inter-session test-retest threshold reliability in a separate group of 50 adult subjects. The ETSPL values for the consumer IE deviated from the reference values for audiometric IEs, with the largest differences (7–9 dB) observed at 500 Hz across ear tips. This is likely related to shallow tip insertions. However, test-retest threshold variations were comparable to those reported for audiometric transducers. Ear tip-specific corrections to the reference thresholds in the standards are required for calibration of consumer IEs used in low-cost audiometry when their ear tips only allow superficial insertion into the ear canal. [ABSTRACT FROM AUTHOR]

Purpose: To review possible risk factors for permanent delayed-onset, progressive sensorineural hearing loss (SNHL) in the paediatric population to recommend follow-up protocols for early detection. Methods: PRISMA-compliant systematic review was performed, including observational studies on the paediatric population up to 16 years old who have passed the newborn hearing screening programme (NHSP), investigating the development of late-onset, progressive SNHL. Electronic searches were performed through Medline, Embase, Cochrane, and Emcare. Results: 37 studies were included. 21 showed an association between late-onset SNHL and congenital cytomegalovirus (cCMV) infection (age at hearing loss diagnosis 0.75 to 204 months, mean 45.6 ± 43.9), while 16 between late-onset SNHL and other congenital or perinatal factors, namely Neonatal Intensive Care Unit (NICU) stay, prematurity, neonatal respiratory failure, mechanical ventilation, extracorporeal membrane oxygenation (ECMO) support, hypocapnia, hypoxia, alkalosis, seizure activity, congenital diaphragmatic hernia (CDH), inner ear malformation, and gene mutations (age at hearing loss diagnosis 2.5 to 156 months, mean 38.7 ± 40.7). Conclusions: cCMV infection may cause late-onset SNHL, which can be missed on standard NHSP. There is, therefore, evidence to support universal screening programmes to enable detection in even asymptomatic neonates. Ongoing audiological follow-up for all children with cCMV is advisable, to enable timely treatment. In the paediatric population presenting conditions such as NICU stay > 5 days, prematurity ≤ 34 weeks gestation, severe neonatal respiratory failure, mechanical ventilation, ECMO support, and CDH surgery, an audiological follow-up from 3 months of age up to at least 3–4 years of age, and at least annually, should be recommended. [ABSTRACT FROM AUTHOR]

Objectives: When there is a difference in hearing on both ears, where to perform the first cochlear implantation (CI) becomes an important issue. The purpose of the study was to evaluate which ear should be chosen for the first implantation in sequential bilateral CI with a long inter-implant period. Methods: The study population consisted of 34 severe-to-profound sensorineural hearing loss pediatrics with the inter-implant period of ≥3 years between the first CI (CI-1) and the second CI (CI-2) before the age of 19 (mean of inter-implant period: 7.1-year). The patients were classified into Group A (CI-1 was performed on the ear with better hearing), Group B (CI-1 on the ear with worse hearing), or Group C (symmetrical hearing in both ears). Speech intelligibility test results were compared between the groups. Results: The monosyllabic word scores of CI-1 were excellent in Groups A (91.7±7.9%) and B (92.5±3.6%) but slightly lower in Group C (85.7±14.9%) before the second implantation (P =.487). At 3 years after the second implantation, all groups demonstrated excellent scores in the bilateral CI condition (95.9±3.0% in Group A; 99.1±.8% in Group B; 97.5±2.9% in Group C, P =.600). However, when the patients were tested in using CI-2 only in Groups A and B after using bilateral CI for 3 years, the scores were inconsistent in Group A (79.6±23.9%; range: 22.2-94.4%), while those were higher and more constant in Group B (92.9±4.8%; 86.8-100.0%). Conclusions: The first CI is strongly recommended to perform on a worse hearing ear if they had different hearing levels between ears. Even with the first CI on a worse hearing ear, its performance never deteriorates. In addition, if they receive the second CI several years later, it will be likely that the second one functions better. [ABSTRACT FROM AUTHOR]

NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids. MR imaging revealed a sizable left frontoparietal, dural-based meningioma with heterogeneous enhancement with mass effect on the brain and midline shift. Multiple additional CNS lesions were noted including a homogenous lesion at the level of T5 indicative of compression of the spinal cord. The patient underwent a frontotemporoparietal craniotomy for the removal of his large dural-based meningioma, utilizing neuronavigation and transdural ultrasonography for precise en bloc resection of the mass. Histopathology revealed an anaplastic meningioma, WHO grade 3, characterized by brisk mitotic activity, small-cell changes, high Ki-67 proliferation rate, and significant loss of P16. We report an anaplastic meningioma associated with an underlying diagnosis of NF2 for which we describe clinical and histopathological features. [ABSTRACT FROM AUTHOR]

Tietz albinism‐deafness syndrome (TADS) is a rare and severe manifestation of Waardenburg syndrome that is primarily linked to mutations in MITF. In this report, we present a case of TADS resulting from a novel c.637G>C mutation in MITF (p.Glu213Gln; GenBank Accession number: NM_000248). A 3‐year‐old girl presented with congenital generalized hypopigmentation of the hair, skin, and irides along with complete sensorineural hearing loss. Histopathological and electron microscopy investigations indicated that this variant did not alter the number of melanocytes in the skin but significantly impaired melanosome maturation within melanocytes. Comprehensive melanin analysis revealed marked reductions in both eumelanin (EM) and pheomelanin (PM) rather than changes in the EM‐to‐PM ratio observed in oculocutaneous albinism. We conducted an electrophoretic mobility shift assay to investigate the binding capability of the identified variant to DNA sequences containing the E‐box motif along with other known variants (p.Arg217del and p.Glu213Asp). Remarkably, all three variants exhibited dominant‐negative effects, thus providing novel insights into the pathogenesis of TADS. This study sheds light on the genetic mechanisms underlying TADS and offers a deeper understanding of this rare condition and its associated mutations in MITF. [ABSTRACT FROM AUTHOR]

Background and Aim: The output Signal-to-Noise Ratio (SNR) is one of the essential factors in hearing aid benefits. There is limited evidence regarding SNR improvement by the Channel-Free (CFHA) and Multi-Channel Hearing Aid (MCHA) and the speech understanding in noise through them. This study aimed to investigate the extent to which output SNR was modified by CFHA and MCHA processing and the variation in aided speech recognition abilities with a change in output SNR. Methods: Thirty-six participants aged 50–65 years were included. A chosen CFHA and MCHA were used to obtain the output SNR and sentence recognition in noise in four different processing algorithms (linear, linear+noise reduction, WDRC, WDRC+noise reduction). Hagerman’s phase inversion technique was used to measure the attenuation of noise and, in turn, to obtain the output SNR of the hearing aid. Results: In all hearing aid processing algorithms among those with normal hearing and people with hearing loss, the output of CFHA revealed higher attenuation values than that of MCHA. There was a significant effect of the hearing aids and processing algorithms in both normal and individuals with hearing impairment on the mean SNR. Further, multiple linear regression analysis results showed that whether the hearing is channel-free or multichannel significantly predicted speech recognition scores, while output SNR and processing algorithms did not. Conclusion: The signal processing algorithms in CFHA had greater noise attenuation values, better output SNR, and speech recognition scores, showing an advantage over the modern MCHA among individuals with hearing impairment. [ABSTRACT FROM AUTHOR]

Background and Aim: Inner ear infection with some viruses may be one of the possible causes of Sensorineural Hearing Loss (SNHL). This study aims to determine the frequency of Herpes Simplex Virus 1 and 2 (HSV-1, HSV-2) and Cytomegalovirus (CMV) in perilymph and peripheral blood samples of cochlear-implanted children. Methods: In this cross-sectional study, 30 children with severe-to-profound SNHL (aged 1.1–5 years) underwent cochlear implantation surgery. During surgery, their perilymph and peripheral blood samples were collected. The samples were analyzed separately for the presence of herpes HSV-1, HSV-2, and CMV by real-time Polymerase Chain Reaction (PCR) method. The load of IgG and IgM antibodies against these viruses was determined using the Enzyme-Linked Immunosorbent Assay (ELISA) method. Results: The frequency of CMV in perilymph samples was 16.7% (5 patients) and in peripheral blood samples was 3.3% (1 patient). The IgG antibody against CMV and HSV1 was positive in 80% and 46.7% of the patients, respectively. The IgM antibody against CMV was positive in 10%. The mean IgM serum antibody load against HSV-1, HSV-2, and CMV was 2.70, 1.70, and 5.47, respectively, and the mean IgG antibody load against these viruses was 56.07, 2.50, and 23.67, respectively. Conclusion: The IgG test is positive in cochlear-implanted children with CMV in their perilymph samples, and the CMV genome is not present in their peripheral blood. This may indicate the previous presence of this virus in the ear and its role in hearing loss. [ABSTRACT FROM AUTHOR]

Background: Glycerophospholipids (GPLs) are essential for cell membrane structure and function. Sphingomyelin and its metabolites regulate cell growth, apoptosis, and stress responses. This study aimed to investigate lipid metabolism in patients experiencing sudden sensorineural hearing loss across all frequencies (AF-SSNHL). Methods: The study included 60 patients diagnosed with unilateral AF-SSNHL, among whom 30 patients had a level of hearing improvement ≥ 15 dB after 6 months of follow-up. A propensity score-matched (2:1) control group was used. Liquid chromatography‒mass spectrometry based untargeted lipidomics analysis combined with multivariate statistics was performed to investigate the lipids change. The "lipidome" R package and weighted gene co-expression network analysis (WGCNA) were utilised to assess the lipids' structural features and the association between lipids and hearing. Results: Lipidomics successfully differentiated the AF-SSNHL group from the control group, identifying 17 risk factors, mainly including phosphatidylcholine (PC), phosphatidylethanolamine (PE), and related metabolites. The ratios of lysophosphatidylcholine/PC, lysophosphatidylethanolamine/PE, and lysodimethylphosphatidylethanolamine/PE were upregulated, while some glycerophospholipid (GPL)-plasmalogens were downregulated in the AF-SSNHL group, indicating abnormal metabolism of GPLs. Trihexosylceramide (d34:1), PE (18:1e_22:5), and sphingomyelin (d40:3) were significantly different between responders and nonresponders, and positively correlated with hearing improvement. Additionally, the results of the WGCNA also suggested that partial GPL-plasmalogens were positively associated with hearing improvement. Conclusion: AF-SSNHL patients exhibited abnormally high blood lipids and pronounced GPLs metabolic abnormalities. Sphingolipids and GPL-plasmalogens had an association with the level of hearing improvement. By understanding the lipid changes, clinicians may be able to predict the prognosis of hearing recovery and personalize treatment approaches. [ABSTRACT FROM AUTHOR]

AbstractObjectivesDesignStudy SampleResultsConclusionsA case of sudden sensorineural hearing loss following use of sildenafil was examined in detail over a period of three days from first report to recovery.Case study. The subject presented with sudden sensorineural hearing loss and diplacusis a day after onset. Testing involved detailed interview, standard audiometry, detailed inter-octave audiometry, and measurement of detailed psychophysical frequency tuning curves during a two day recovery period.One male aged in his thirties with otherwise normal hearing.Although standard audiometry was within normal limits, detailed inter-octave audiometry and psychophysical frequency tuning curves were consistent with a punctate unilateral intra-cochlear lesion that resolved over a period of three days.This is the first report of such a frequency-specific audiometric shift and diplacusis after sildenafil, and is not consistent with previous reports of direct ototoxic pharmacological effects. We propose that the lesion was most likely caused by a cochlear bleed, and may have been due to physical exertion rather than a direct pharmaceutical effect. The study highlights the important role of additional diagnostic testing that can be easily achieved in a clinical setting with minimal equipment [ABSTRACT FROM AUTHOR]

Background: Townes-Brocks syndrome (TBS) is a rare genetic disorder characterized by imperforate anus, dysplastic ears, thumb malformations, and other abnormalities. Previous studies have revealed that mutations in the SALL1 gene can disrupt normal development, resulting in the characteristic features of Townes-Brocks syndrome. Spalt-like transcription factors (SALLs) are highly conserved proteins that play important roles in various cellular processes, including embryonic development, cell differentiation, and cell survival. Over 400 different variants or mutations have been reported in the SALL1 gene in individuals with TBS. Most of these variants lead to the formation of premature termination codons (PTCs), also known as nonsense mutations. The majority of these PTCs occur in a specific region of the SALL1 gene called the "hotspot region", which is particularly susceptible to mutation. Methods: In this study, we conducted whole-exome sequencing on a three-generation Chinese family with anorectal malformations. Results: We identified a novel heterozygous mutation (chr16:51175376:c.757 C > T p.Gln253*) in the SALL1 gene. Molecular analysis revealed a heterozygous C to T transition at nucleotide position 757 in exon 2 of the SALL1 (NM_002968) gene. This mutation is predicted to result in the substitution of the Gln253 codon with a premature stop codon (p.Gln253*). The glutamine-rich domain forms a long alpha helix, enabling the mutant protein to interact with the wild-type SALL1 protein. This interaction may result in steric hindrance effects on the wild-type SALL1 protein. Conclusions: Our findings have expanded the mutation database of the SALL1 gene, which is significant for genetic counseling and clinical surveillance in the affected family. Furthermore, our study enhances the understanding of Townes-Brocks syndrome and has the potential to improve its diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Background: Sensory information obtained from the visual, somatosensory, and vestibular systems is responsible for regulating postural control, and if damage occurs in one or more of these sensory systems, postural control may be altered. Objective: To evaluate and compare the postural sway velocity between children with normal hearing and with sensorineural hearing loss (SNHL), matched by sex and age group, and to compare the postural sway velocity between children with normal hearing and with SNHL, with and without vestibular dysfunction. Methods: Cross-sectional study that evaluated 130 children (65 with normal hearing and 65 with SNHL), of both sexes and aged between 7 and 11 years, from public schools of the city of Caruaru, Pernambuco state, Brazil. The postural sway velocity of the center of pressure (COP) was assessed by a force platform, in two directions, anteroposterior (AP) and mediolateral (ML)), in three positions, namely bipedal support with feet together and parallel (parallel feet (PF)), bipedal support with one foot in front of the other (tandem foot (TF)), and single-leg support (one foot (OF)), evaluated with the eyes open and closed. Results: Children with SNHL demonstrated greater postural sway velocity compared to children with normal hearing in all the positions evaluated, with significant differences in the AP direction, with the eyes open (PF: p = 0.001; TF: p = 0.000; OF: p = 0.003) and closed (PF: p = 0.050; TF: p = 0.005). The same occurred in the ML direction, with the eyes open (PF: p = 0.001; TF: p = 0.000; OF: p = 0.001) and closed (PF: p = 0.002; TF: p = 0.000). The same occurred in relation to vestibular function, where the children with SNHL with an associated vestibular dysfunction demonstrated greater postural sway velocity compared to children with normal hearing in all the positions evaluated, demonstrating significant differences in the AP direction, with the eyes open (TF: p = 0.001; OF: p = 0.029) and eyes closed (PF: p = 0.036; TF: p = 0.033). The same occurred in the ML direction, with the eyes open (TF: p = 0.000) and with the eyes closed (PF: p = 0.008; TF: p = 0.009). Conclusions: Children with SNHL demonstrated greater instability of postural control than children with normal hearing in all the directions assessed. Children with SNHL and an associated vestibular dysfunction demonstrated the greatest instability of postural control in this study. [ABSTRACT FROM AUTHOR]

Background: Accurate prognostic prediction is crucial for managing Idiopathic Sudden Sensorineural Hearing Loss (ISSHL). Previous studies developing ISSHL prognosis models often overlooked individual variability in hearing damage by relying on fixed frequency domains. This study aims to develop models predicting ISSHL prognosis one month after treatment, focusing on patient-specific hearing impairments. Methods: Patient-Personalized Seigel's Criteria (PPSC) were developed considering patient-specific hearing impairment related to ISSHL criteria. We performed a statistical test to assess the shift in the recovery assessment when applying PPSC. The utilized dataset of 581 patients comprised demographic information, health records, laboratory testing, onset and treatment, and hearing levels. To reduce the model's reliance on hearing level features, we used only the averages of hearing levels of the impaired frequencies. Then, model development, evaluation, and interpretation proceeded. Results: The chi-square test (p-value: 0.106) indicated that the shift in recovery assessment is not statistically significant. The soft-voting ensemble model was most effective, achieving an Area Under the Receiver Operating Characteristic Curve (AUROC) of 0.864 (95% CI: 0.801–0.927), with model interpretation based on the SHapley Additive exPlanations value. Conclusions: With PPSC, providing a hearing assessment comparable to traditional Seigel's criteria, the developed models successfully predicted ISSHL recovery one month post-treatment by considering patient-specific impairments. [ABSTRACT FROM AUTHOR]

IntroductionCase ReportConclusionIn addition to sensorineural hearing loss, Waardenburg Syndrome (WS) may present with variable pigmentation of skin and choroid, which may simulate other life-threating conditions (e.g. melanoma).Two siblings ostensibly presented with unilateral choroidal pigmentary abnormalities concerning for choroidal tumour. Serial ophthalmic examination documented no lesion growth (base or height) whilst the apparent syndromic features (i.e. iris hypochromia, profound sensorineural hearing loss, SNHL), family history (autosomal dominant inheritance) and positive genetic testing (pathogenic MITF variant) led to a revised diagnosis of Waardenburg Syndrome type 2A.Sectoral preservation of choroidal pigmentation in WS is rarely associated with choroidal malignancy. Awareness of syndromic features (e.g. SNHL) and access to genetic testing may facilitate early accurate diagnosis (i.e. allay concern for malignancy), enable treatment of modifiable features (e.g. SNHL) and identify other affected relatives. [ABSTRACT FROM AUTHOR]

Background: Previous studies have implicated inherited mutations in mitochondrial DNA (mtDNA) in sensorineural hearing loss (SNHL). However, the definitive association between mitochondrial 12S rRNA (MT-RNR1) variants and hearing loss in the population has not been well established, particularly in Asia. The objective of this retrospective cohort study was to assess the association between MT-RNR1 variants and the risk of SNHL in patients in Taiwan. Methods: The cohort included 306,068 participants from Taiwan between January 2003 and December 2020. Participants were classified based on genetic variants, particularly mitochondrial mutations (rs267606618, rs267606619, rs267606617). MT-RNR1 variant cases were matched 1:10 with non-mutant patients by age, gender, and visit year, excluding those with pre-existing hearing loss. The primary endpoint was SNHL, identified using specific ICD-TM codes with a 90% positive predictive value. Medication exposure history was determined via self-report or electronic medical records in the hospital. Cox proportional hazard regression models were used to assess the association between MT-RNR1 variants and hearing loss, adjusting for various covariates. Kaplan-Meier survival curves and log-rank tests compared hearing loss incidence between groups. Results: The mean age of the mtDNA variants group is 32.4 years, with a standard deviation of 19.2 years. The incidence density of hearing loss for the mutation group was 36.42 per 10,000 person-years (95% Confidence Interval [CI], 27.21–47.73), which was higher than the 23.77per 10,000 person-years (95% CI, 21.32–26.42) in the wild-type group (p = 0.0036). Additionally, diabetes mellitus was associated with an increased risk of developing SNHL in individuals with MT-RNR1 variants (adjusted hazard ratio = 1.76 [95% CI, 1.00-3.09], p < 0.05). Conclusion: This study highlights the increased risk of hearing loss in patients carrying MT-RNR1 variants, particularly those with diabetes mellitus. Future research that integrates genetic and clinical data is crucial for developing more precise interventions to monitor and treat hearing loss in this vulnerable population. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study was to investigate the hearing characteristics and causes of sudden sensorineural hearing loss (SSNHL) in patients aged from 15 to 40 years, focusing on audiological outcomes one year after the diagnosis. Methods: The medical records of individuals with SSNHL who were referred to our tertiary-level audiologic center were reviewed. All patients had undergone comprehensive diagnostic evaluations, including high-resolution 3D-FLAIR delayed magnetic resonance imaging (MRI), cone beam computed tomography (CBCT), and screening for coagulation, infectious, and autoimmune diseases. Results: Overall, 56 patients (mean age 28.1 ± 7.6 years) were included in the study. The hearing threshold in the affected ear improved significantly from 56.0 ± 18.0 dB at the diagnosis to 46.9 ± 22.3 dB after one year (p = 0.02). The degree of hearing loss, audiometric configurations, hearing improvements, and adherence to hearing treatments showed considerable variability among patients. Aural fullness, tinnitus, and hyperacusis were the predominant symptoms associated with SSNHL, and their prevalence decreased significantly over time. The diagnostic protocol led to the identification of the specific cause of SSNHL in 75% (42/56) of patients. The known etiology was found to be otological (39.3%), infectious (21.4%), autoimmune (7.1%), vascular (5.4%), or neoplastic (1.8%). In particular, Menière's disease (n = 12), isolated cochlear endolymphatic hydrops (n = 6), HSV-1 (n = 5), and EBV (n = 4) infections were the most frequent causes of SSNHL. Conclusions: The identification of the specific etiology of SSNHL may facilitate a more personalized approach to management and treatment. [ABSTRACT FROM AUTHOR]

Objectives: Simultaneous removal and cochlear implantation (CI) have been reported in intralabyrinthine and intracochlear schwannoma. A wide range of postoperative hearing outcomes have been reported after CI in these cases. This study evaluated the outcomes of performing a simultaneous resection of Schwannoma in cochlea and cochlear implantation (CI), aiming to assess the effectiveness of this combined surgical approach for hearing rehabilitation with CI. Methods: This retrospective case series was conducted at a tertiary care center. The study included four consecutive patients with profound sensorineural hearing loss due to a mass inside the cochlea. These patients underwent simultaneous single-sided CI and tumor resection performed by the same surgeon. Preoperative and postoperative audiological assessments were conducted to evaluate the patients' hearing outcomes before and after the surgical intervention. Results: Simultaneous CI with tumor resection was successful in all cases. Two of the four patients had a unilateral tumor, while the other two had a bilateral tumor with the involvement of the internal auditory canal and cerebellopontine angle (neurofibromatosis type 2 (NF2)). In two cases of unilateral tumor, aided free-field pure tone average (PTA) was 26 dB, and 46 dB hearing level (HL), and word recognition score (WRS) at 65 dB was 40% and 68%, respectively, 3 months after surgery. In two cases of tumor with NF2, aided free-field PTA was 36 dB and 60 dB HL, and both cases showed 0% WRS at 65 dB 3 months after surgery. Conclusions: Simultaneous schwannoma excision and CI in patients with Schwannoma inside cochlea are surgically practical and safe. Postoperatively, there was a notable improvement in hearing in cases of sporadic schwannoma, regardless of the type of CI used. However, there was 0% WRS in the two NF2 patients with a mass in the internal auditory canal. [ABSTRACT FROM AUTHOR]

Objective: Studies evaluating genetic sensorineural hearing loss (SNHL) in Hispanic and Latino populations using genomic technologies are lacking. Recent data has shown that Hispanic and Latino children display lower genetic diagnostic rates despite similar prevalence rates of SNHL to their Asian and White counterparts, thus negatively affecting their clinical care. Our objective was to determine the genetic contribution to SNHL in a population of Mexican children undergoing evaluation for cochlear implantation. Methods: Pediatric patients from Mexico with severe to profound SNHL undergoing evaluation for cochlear implantation were recruited. Exome sequencing (ES) or hearing loss gene panel testing was performed. Variant pathogenicity was established in accordance to criteria established by the American College of Medical Genetics, and variants of interest were clinically confirmed via CLIA certified laboratory. Results: Genetic evaluation was completed for 30 Mexican children with severe to profound SNHL. A genetic cause was identified for 47% (14) of probands, and 7% (2) probands had an inconclusive result. Of the diagnoses, 10 (71%) were syndromic or likely syndromic, and 4 (29%) were nonsyndromic. Eight probands (80% of all syndromic diagnoses) were diagnosed with a syndromic form of hearing loss that mimics a nonsyndromic clinical presentation at a young age and so could not be suspected based on clinical evaluation alone without genetic testing. Conclusion: This is the largest study to date to use comprehensive genomic testing for the evaluation of Mexican children with severe to profound SNHL. A significant proportion of children in this cohort were diagnosed with syndromic hearing loss. Future study in a larger cohort of Mexican children with varying degrees of hearing loss is required to improve the efficacy of genetic testing and timely medical intervention within these ethnically diverse populations. [ABSTRACT FROM AUTHOR]

Background: Otosclerosis is characterized by abnormal bone growth in the otie capsule. Nowadays, stapedotomy is commonly used for otosclerosis treatment. Currently, postoperative dizziness has been associated with stapedotomy. In 1981, intratympanic dexamethasone was utilized to manage inner ear disorders like tinnitus and acute sensorineural hearing loss. However, there is much uncertainty regarding the effect and safety of topical steroid therapy in the middle ear during stapedotomy. In the present study, we assessed the effect of topical steroid therapy during stapedotomy on postoperative dizziness. Methods: Otosclerosis patients eligible for stapedotomy were randomly divided into two groups based on dexamethasone administration or placebo. Audiometric and tympanometry results were observed and recorded for the involved frequencies. The Dizziness Handicap Inventory was used to quantify patient perceptions of dizziness and balance issues. Audiometry and dizziness assessments were repeated at discharge and 4 months after the operation. Results: The study comprised 72 otosclerosis patients undergoing stapedotomy. At discharge, the intervention group showed a significant reduction in the incidence of dizziness compared to the placebo group. However, in the 4-month follow-up after the operation, both groups experienced a decrease in dizziness incidence, with no significant difference between them. There was also no significant difference in audiometric levels between the two groups. Interestingly, the intervention group had a significantly lower need for systemic anti-dizziness drugs after surgery compared to the control group. Conclusion: Topical dexamethasone during stapedotomy effectively minimizes dizziness at discharge and reduces the need for postoperative anti-dizziness medication. [ABSTRACT FROM AUTHOR]

Objectives: To establish audiological and other outcomes following cochlear implantation in humans and animals with eluting electrodes. Methods: Systematic review and narrative synthesis. Databases searched (April 2023): MEDLINE, EMBASE, CENTRAL, ClinicalTrials.gov, and Web of Science. Studies reporting outcomes in either humans or animals following cochlear implantation with a drugeluting electrode were included. No limits were placed on language or year of publication. Risk of bias assessment was performed on all included studies using either the Brazzelli or Systematic Review Centre for Laboratory animal Experimentation (SYRCLE) assessment tools. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 statement. Results: Searches identified 146 abstracts and 108 full texts. Of these, 18 studies met the inclusion criteria, reporting outcomes in 523 animals (17 studies) and 24 humans (1 study). Eluting electrodes included dexamethasone (16 studies), aracytine (1 study), nicotinamide adenine dinucleotide (1 study), the growth factors insulinlike growth factor 1 (IGF1) and hepatocyte growth factor (HGF) (1 study), and neurotrophin-3 (1 study). All included studies compare outcomes following implantation with an eluting electrode with a control non-eluting electrode. In the majority of studies, audiological outcomes (e.g., auditory brainstem response threshold) were superior following implantation with an eluting electrode compared with a standard electrode. Most studies which investigated post-implantation impedance reported lower impedance following implantation with an eluting electrode. The influence of eluting electrodes on other reported outcomes (including post-implantation cochlear fibrosis and the survival of hair cells and spiral ganglion neurons) was more varied across the included studies. Conclusions: Eluting electrodes have shown promise in animal studies in preserving residual hearing following cochlear implantation and in reducing impedance, though data from human studies remain lacking. Further in-human studies will be required to determine the clinical usefulness of drug-eluting cochlear implants as a future treatment for sensorineural hearing loss. [ABSTRACT FROM AUTHOR]

Significance Statement: Primary middle ear inverted papilloma is an exceedingly rare finding posing a diagnostic challenge, as symptomatology often mimics common clinical entities like serous otitis media. Clinical and radiological findings are not specific, whilst high recurrence rates, local destruction, and potential malignant transformation constitute an overall demanding surgical challenge, requiring aggressive procedures. We report the case of a primary inverted papilloma of the middle ear, discussing diagnostic and therapeutic issues. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to assess degree of audiovestibular handicap in patients with vestibular schwannoma. Methods: Audiovestibular handicap was assessed using the Hearing Handicap Inventory, Tinnitus Handicap Inventory and Dizziness Handicap Inventory. Patients completed questionnaires at presentation and at least one year following treatment with microsurgery, stereotactic radiosurgery or observation. Changes in audiovestibular handicap and factors affecting audiovestibular handicap were assessed. Results: All handicap scores increased at follow up, but not significantly. The Tinnitus Handicap Inventory and Dizziness Handicap Inventory scores predicted tinnitus and dizziness respectively. The Hearing Handicap Inventory was not predictive of hearing loss. Age predicted Tinnitus Handicap Inventory score and microsurgery was associated with a deterioration in Dizziness Handicap Inventory score. Conclusion: Audiovestibular handicap is common in patients with vestibular schwannoma, with 75 per cent having some degree of handicap in at least one inventory. The overall burden of handicap was, however, low. The increased audiovestibular handicap over time was not statistically significant, irrespective of treatment modality. [ABSTRACT FROM AUTHOR]

Aim: Biallelic mutations in the PTRH2 gene have been associated with infantile multisystem neurological, endocrine, and pancreatic disease (IMNEPD), a rare autosomal recessive disorder of variable expressivity characterized by global developmental delay, intellectual disability or borderline IQ level, sensorineural hearing loss, ataxia, and pancreatic insufficiency. Various additional features may be included, such as peripheral neuropathy, facial dysmorphism, hypothyroidism, hepatic fibrosis, postnatal microcephaly, cerebellar atrophy, and epilepsy. Here, we report the first Italian family presenting only predominant neurological features. Methods: Extensive neurological and neurophysiological evaluations have been conducted on the two affected brothers and their healthy mother since 1996. The diagnosis of peripheral neuropathy of probable hereditary origin was confirmed through a sural nerve biopsy. Exome sequencing was performed after the analysis of major neuropathy‐associated genes yielded negative results. Results: Whole‐exome sequencing analysis identified the homozygous substitution c.256C>T (p.Gln86Ter) in the PTRH2 gene in the two siblings. According to American College of Medical Genetics and Genomics (ACMG) guidelines, the variant has been classified as pathogenic. At 48 years old, the proband's reevaluation confirmed a demyelinating sensorimotor polyneuropathy with bilateral sensorineural hearing loss that had been noted since he was 13. Additionally, drug‐resistant epileptic seizures occurred when he was 32 years old. No hepatic or endocrinological signs developed. The younger affected brother, 47 years old, has an overlapping clinical presentation without epilepsy. Interpretation: Our findings expand the clinical phenotype and further demonstrate the clinical heterogeneity related to PTRH2 variants. We thereby hope to better define IMNEPD and facilitate the identification and diagnosis of this novel disease entity. [ABSTRACT FROM AUTHOR]

Objectives Nucleotide-binding oligomerization domain-like receptor family, pyrin domain containing 3-associated autoinflammatory disease (NLRP3 -AID) is a rare autosomal dominant systemic autoinflammatory disease. We aimed to summarize the phenotypic and genotypic features of Chinese adult NLRP3 -AID patients with hearing loss. Methods A retrospective cohort study of twenty-one adult patients with NLRP3 -AID was conducted in Peking Union Medical College Hospital between July 2015 and March 2023. All patients underwent whole exome sequencing and otorhinolaryngologic assessments. Clinical features and therapeutic data were collected and analysed. Results We found that 13/21 (61.90%) of patients had hearing loss with high-frequency impairment in the majority, and most patients presented with vestibular dysfunction as a new finding. The NLRP3 -AID patients with early-onset, cold or stress triggered episodes, red eyes, fatigue, hypopsia and mutations located in the NACHT domain of the NLRP3 protein were more likely to suffer from hearing loss, especially sensorineural hearing loss, perhaps as a result of pathogenic variants of high penetrance. By a series of audiological evaluations, TNF-α inhibitors were confirmed to improve or reverse hearing loss. Conclusions We reported the first cohort of Chinese adult NLRP3 -AID patients with hearing loss and characterized vestibular dysfunction, highlighted the necessity for attention to high-frequency hearing and provided potential alternative treatment. [ABSTRACT FROM AUTHOR]

Since the issuance of the emergency use authorization (EUA) of 3 coronavirus disease 2019 (COVID-19) vaccines, there have been over 180 million individuals fully vaccinated in the United States (US). With the increasing administration of COVID-19 vaccinations, there have been over 550,000 adverse events reported in the Vaccine Adverse Event Reporting System (VAERS) with approximately 230,000 experienced after receipt of the Pfizer-BioNTech COVID-19 Vaccine as of September 23rd 2021. Audio-vestibular symptoms (including Sudden Sensorineural Hearing Loss (SSNHL)) secondary to immunizations has previously been evaluated. However, this report describes the first case of bilateral sudden sensorineural hearing loss potentially due to the Pfizer-BioNTech COVID-19 vaccine. We further review the available literature regarding the treatment of Sudden Sensorineural Hearing Loss, and the association of SSNHL with previous immunizations and COVID-19 infection. Lastly, we hypothesize the underlying potential mechanisms between SSNHL and the Pfizer-BioNTech COVID-19 vaccine. [ABSTRACT FROM AUTHOR]

Objective: To compare white matter hyperintensities (WMHs) on T2-weighted magnetic resonance imaging (MRI) of patients with sudden sensorineural hearing loss (SSNHL) and analyze subpopulations with age-matched controls. Methods: T2-weighted MRI scans of 150 patients with SSNHL were assessed for WMHs and compared with the data of 148 healthy age-matched adults. Assessments of WMHs included independent grading of deep white matter hyperintensities (DWMHs) and periventricular hyperintensities (PVHs). WMH severity was visually rated using the Fazekas and Mirsen scales by two independent observers. Results: Fazekas grades for PVHs (p < 0.001) and DWMHs (p < 0.001) of SSNHL patients were found to be significantly greater than those of healthy participants. The average Mirsen grades for DWMHs of healthy and SSNHL patients were evaluated to be 0.373 ± 0.550 and 2.140 ± 0.859, respectively. Mirsen grades for DWMHs of SSNHL patients were found to be significantly greater (p < 0.001) than those of healthy participants. The Mirsen scale was found to have higher sensitivity (p < 0.001) than the Fazekas scale in grading PVHs and DWMHs. No significant difference (p = 0.24) was found in specificities between the two scales. Conclusions: Patients with sudden hearing loss have a much higher likelihood of having periventricular and deep white matter hyperintensities compared to age-matched controls. These findings indicate that sudden hearing loss patients are more likely to have microvascular changes in the brain, which may indicate a vascular and/or migraine origin to sudden sensorineural hearing loss. [ABSTRACT FROM AUTHOR]

Introduction: Mastoidectomy is a surgical procedure that removes infection, along with cholesteatoma, from the mastoid and middle ear. Cholesteatoma, a progressive disease that erodes bone when left untreated, leads to complications. Aim: To ascertain the most common mastoidectomy-related intraoperative and postoperative complications. Materials and Methods: This study was a retrospective descriptive study of patients who underwent mastoidectomy over one year at a tertiary care hospital in Assam, India between April 2022 to March 2023. All the patients planned for mastoidectomy were evaluated for intraoperative complications like facial nerve injury or postoperative complications immediately after the postoperative period (e.g., wound dehiscence) or between 1-3 months (e.g., Sensorineural Hearing Loss (SNHL), dead ear, etc.). The data collected was tabulated in a Microsoft Excel Worksheet, and the categorical variables were summarised as proportions and percentages. Results: A total of 62 patients underwent mastoidectomy and were reviewed over the one-year period retrospectively. Out of these, 28 (45.16%) developed complications. Two out of 62 patients (3.2%) experienced facial nerve injury intraoperatively. One patient (1.6%) with a high jugular bulb experienced massive bleeding intraoperatively while raising the tympanomeatal flap. Labyrinthine injury, dural plate injury, and sigmoid sinus injury were each found in one patient (1.6%). Eight out of 62 patients (12.9%) developed persistent otorrhea. Additionally, five patients (8%) had wound dehiscence and were treated with antibacterials, antiseptic dressing, and secondary suturing when needed. Two patients (3.2%) experienced SNHL; preoperatively, they had mild conductive hearing loss which postoperatively converted to mild to moderate mixed hearing loss. Postoperative dead ear was observed in one patient (1.6%). There was one patient (1.6%) with an unfavourably displaced pinna, and two patients (3.2%) had recurrent cholesteatoma. Vertigo was seen in two cases of radical mastoidectomy and one case of modified radical mastoidectomy. Conclusion: With proper planning and sound surgical techniques, avoidance of mastoidectomy complications can be achieved. [ABSTRACT FROM AUTHOR]

Background: Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a maternally inherited mitochondrial disease that affects various systems in the body, particularly the brain, nervous system, and muscles. Among these systems, sensorineural hearing loss is a common additional symptom. Methods: A 42-year-old female patient with MELAS who experienced bilateral profound deafness and underwent bilateral sequential cochlear implantation (CIs). Speech recognition and subjective outcomes were evaluated. Results: Following the first CI follow-up, the patient exhibited improved speech recognition ability and decided to undergo the implantation of the second ear just two months after the initial CI surgery. The second CI also demonstrated enhanced speech recognition ability. Subjective outcomes were satisfactory for bilateral CIs. Conclusions: MELAS patients receiving bilateral CIs can attain satisfactory post-CI speech recognition, spatial hearing, and sound qualities. [ABSTRACT FROM AUTHOR]

Purpose: Patient-to-image registration is a preliminary step required in surgical navigation based on preoperative images. Human intervention and fiducial markers hamper this task as they are time-consuming and introduce potential errors. We aimed to develop a fully automatic 2D registration system for augmented reality in ear surgery. Methods: CT-scans and corresponding oto-endoscopic videos were collected from 41 patients (58 ears) undergoing ear examination (vestibular schwannoma before surgery, profound hearing loss requiring cochlear implant, suspicion of perilymphatic fistula, contralateral ears in cases of unilateral chronic otitis media). Two to four images were selected from each case. For the training phase, data from patients (75% of the dataset) and 11 cadaveric specimens were used. Tympanic membranes and malleus handles were contoured on both video images and CT-scans by expert surgeons. The algorithm used a U-Net network for detecting the contours of the tympanic membrane and the malleus on both preoperative CT-scans and endoscopic video frames. Then, contours were processed and registered through an iterative closest point algorithm. Validation was performed on 4 cases and testing on 6 cases. Registration error was measured by overlaying both images and measuring the average and Hausdorff distances. Results: The proposed registration method yielded a precision compatible with ear surgery with a 2D mean overlay error of 0.65 ± 0.60 mm for the incus and 0.48 ± 0.32 mm for the round window. The average Hausdorff distance for these 2 targets was 0.98 ± 0.60 mm and 0.78 ± 0.34 mm respectively. An outlier case with higher errors (2.3 mm and 1.5 mm average Hausdorff distance for incus and round window respectively) was observed in relation to a high discrepancy between the projection angle of the reconstructed CT-scan and the video image. The maximum duration for the overall process was 18 s. Conclusions: A fully automatic 2D registration method based on a convolutional neural network and applied to ear surgery was developed. The method did not rely on any external fiducial markers nor human intervention for landmark recognition. The method was fast and its precision was compatible with ear surgery. [ABSTRACT FROM AUTHOR]

To evaluate the potential impact of coronavirus disease 2019 (COVID-19) and vaccinations on otologic diseases, including facial nerve paralysis (including Ramsay Hunt syndrome), vestibular neuritis, sudden sensorineural hearing loss, and Meniere's disease. In this retrospective study, we conducted a time-series analysis employing a causal impact algorithm on a large-scale inpatient database in Japan. We compared the actual number of hospitalized patients with otologic diseases to two predictions: one without any covariates and another with a covariate accounting for the reduction in the number of hospitalized patients due to lockdown measures. Additionally, we performed Granger causality tests to ensure the robustness of our findings. No significant increase was noted in the number of hospitalized patients with otologic diseases following the onset of the COVID-19 pandemic in the causal impact analysis. Similarly, no notable surge was observed in hospitalizations for these diseases following the introduction of the COVID-19 vaccine. The Granger causality tests results aligned with the causal impact analysis findings. Our findings indicate that COVID-19 and vaccinations had minimal discernible effects on hospitalization of patients with otologic diseases, suggesting that otologic diseases may not be significantly impacted by COVID-19 and vaccinations, which could have implications for public health policies and the allocation of healthcare resources during a pandemic. Further research and monitoring of long-term effects are warranted to validate these findings and guide healthcare decision-making. [ABSTRACT FROM AUTHOR]

Cholesteatoma secondary to tympanic perforation, known as "secondary acquired cholesteatoma" may progress slower than a retraction pocket cholesteatoma, with less bone destruction and fewer intracranial complications. However, complete surgical removal remains difficult because the pathological epithelium on the marginal side of the extension is not covered by the subepithelial layer of the cholesteatoma matrix, making the boundary with the middle ear mucosa difficult to identify. Therefore, considering the pathophysiology of secondary acquired cholesteatoma, suitable preoperative evaluation and surgical techniques are required. In this study, we aimed to evaluate (i) the extension of secondary acquired cholesteatoma according to the size and location of tympanic membrane perforation; and (ii) the microscopic surgical outcomes, including the rate of residual cholesteatoma, requirement for specialized surgical management, and changes in hearing. This retrospective study included data of cases with secondary acquired cholesteatoma (n = 66; 66 ears of 64 patients, 2 patients had bilateral ear involvement), including those who underwent a staged operation (n = 25). The perforation level of the tympanic membrane was associated with the cholesteatoma extension. When the cholesteatoma extended around the stapes, staged operation was chosen. Six cases of spontaneous resolution of stapes lesions at the time of staged surgery were observed. A significant postoperative improvement in hearing was observed; however, five cases experienced sensorineural hearing loss. Surgery, including staged surgery of the stapes lesions, along with careful observation of the perforation, is required in secondary acquired cholesteatoma. For lesions that are visible yet challenging to remove, it is imperative to exercise prudent judgment, taking into account the possibility of spontaneous resolution. [ABSTRACT FROM AUTHOR]

Simple Summary: The hallmark of NF2 is bilateral vestibular schwannomas, which progressively enlarge, leading to sensorineural hearing loss, tinnitus, facial weakness, and pain that translates to social impairment and clinical depression. To better understand disease progression and characterize treatment response, we developed a panel of five tests suitable for the mouse vestibular schwannoma model and investigated how tumor growth and treatment affect gait, coordination, and motor function. These methods, paired with hearing tests, enable a comprehensive evaluation of tumor-induced neurological deficits and facilitate the assessment of the effectiveness of novel therapeutics to improve NF2 treatments. NF2-related Schwannomatosis (NF2-SWN) is a disease that needs new solutions. The hallmark of NF2-SWN, a dominantly inherited neoplasia syndrome, is bilateral vestibular schwannomas (VSs), which progressively enlarge, leading to sensorineural hearing loss, tinnitus, facial weakness, and pain that translates to social impairment and clinical depression. Standard treatments for growing VSs include surgery and radiation therapy (RT); however, both carry the risk of further nerve damage that can result in deafness and facial palsy. The resultant suffering and debility, in combination with the paucity of therapeutic options, make the effective treatment of NF2-SWN a major unmet medical need. A better understanding of these mechanisms is essential to developing novel therapeutic targets to control tumor growth and improve patients' quality of life. Previously, we developed the first orthotopic cerebellopontine angle mouse model of VSs, which faithfully mimics tumor-induced hearing loss. In this model, we observed that mice exhibit symptoms of ataxia and vestibular dysfunction. Therefore, we further developed a panel of five tests suitable for the mouse VS model and investigated how tumor growth and treatment affect gait, coordination, and motor function. Using this panel of ataxia tests, we demonstrated that both ataxia and motor function deteriorated concomitantly with tumor progression. We further demonstrated that (i) treatment with anti-VEGF resulted in tumor size reduction, mitigated ataxia, and improved rotarod performance; (ii) treatment with crizotinib stabilized tumor growth and led to improvements in both ataxia and rotarod performance; and (iii) treatment with losartan did not impact tumor growth nor ameliorate ataxia or motor function. Our studies demonstrated that these methods, paired with hearing tests, enable a comprehensive evaluation of tumor-induced neurological deficits and facilitate the assessment of the effectiveness of novel therapeutics to improve NF2 treatments. [ABSTRACT FROM AUTHOR]

Sudden deafness poses a significant threat to patients' quality of life, yet effective indicators for evaluating its onset and prognosis remain elusive. The inner ear is primarily supplied by the labyrinthine artery, which lacks collateral circulation. Changes in coagulation function and hemorheology can cause spasm or thrombosis of the labyrinthine artery, leading to ischemia, hypoxia, and microcirculation disorders in the inner ear, ultimately resulting in sudden deafness. This retrospective study examined 196 patients with sudden deafness, utilizing the 2015 Chinese guideline for diagnosis and treatment classification. Coagulation system analysis used the STA-R Evolution automatic coagulation analyzer, measuring activated partial thromboplastin time (APTT), prothrombin time (PT), and fibrinogen (FIB). Plasma lactate concentration was determined using a Johnson and Johnson Fusion 5.1 model plasma lactate detector. Results of the study revealed a correlation between the degree of hearing loss and disease prognosis. Patients with higher grade hearing loss exhibited elevated plasma lactate levels upon admission compared with those with lower grade hearing loss. Importantly, elevated plasma lactate levels at admission served as predictive indicators for treatment outcomes. In addition, patients with ineffective treatment demonstrated a more coagulable blood state, as evidenced by the lower APTT (ineffective treatment: 31.47 ± 4.55 seconds, effective treatment: 35.17 ± 5.38 seconds) and PT on admission, but higher plasma FIB. In conclusion, plasma lactate levels upon admission hold promise as prognostic markers for sudden deafness treatment outcomes, providing valuable insights for clinical management. [ABSTRACT FROM AUTHOR]

Background: Age-related hearing loss (ARHL) is a general term used to describe the sensorineural type of hearing loss occurring in both ears in older adults. Neurotrophins are the most promising candidates for supporting the auditory nerve by increasing neuronal survival. This study aimed to help elucidate the pathophysiology of ARHL by determining whether any relationship exists between brain-derived neurotrophic factor (BDNF) and neurotrophin-3 (NT-3) levels in serum samples from patients diagnosed with ARHL. Materials and Method: Seventy-seven individuals, a study group of 41 patients diagnosed with ARHL, and a control group of 36 participants without hearing loss were evaluated. Serum samples were collected and used to measure serum BDNF and NT-3 levels with the new Nepenthe enzyme-linked immunosorbent assay method. Results: Median pure-tone average results in the 2000, 4000, and 6000 Hz ranges were 52.5 (44.3–67.3) dB HL in the ARHL group and 13.5 (11.1–17.1) dB HL in the control group. The difference was statistically significant (p = .001). Although NT-3 and BDNF levels were both lower in ARHL patients than in participants without hearing loss, only the BDNF levels were significantly (p = .002) lower. Mean left and right ear word recognition scores were also lower in ARHL patients than in control groups. The ARHL group was further divided into two subgroups based on word recognition scores to evaluate significant differences in BDNF and NT-3 levels. No statistically significant difference was observed in BDNF and NT-3 levels between these subgroups. However, there was a significant difference in word recognition scores. Conclusions: Low BDNF levels in the ARHL group suggest that BDNF may play a role in the pathogenesis of ARHL. Patients with low (ARHL1) and high (ARHL2) word recognition scores were compared for the first time in the literature in terms of BDNF and NT-3 levels. However, the results were not statistically significant. This article is a preliminary study and was written to provide guidance for our next comprehensive project. [ABSTRACT FROM AUTHOR]

Objective:Methods:Results:Conclusion:This study emphasizes the role of preoperative high-resolution computed tomography (HRCT) temporal bone in evaluating the variation in cochlear orientation and proposes a grading system to determine the level of intraoperative difficulties encountered.Preoperative correlation of middle ear and inner ear structures along with the basal turn angle (BTA) was done to assess the orientation. Patients were divided into three groups depending on BTA and radiology findings.Group A (BTA = 55°-60°) had statistically significant (p < 0.05) correlation between middle ear to inner ear structures on HRCT, whereas group B (BTA >60°) and C (BTA <55°) had variations in the alignment of middle ear structures to their corresponding inner ear structures.The association of BTA to the correlation between middle ear and inner ear structures can determine the orientation of the cochlea. This evaluation makes the surgeon aware of intraoperative challenges and helps in surgical planning. [ABSTRACT FROM AUTHOR]

Background: Mutations in the GJB2 gene, which encodes the protein connexin 26 and is involved in inner ear homeostasis, are identified in approximately 50% of patients with autosomal recessive nonsyndromic hearing loss, making it one of the primary causes of prelingual nonsyndromic hearing loss in various populations. The 35delG mutation, one of the most common mutations of the GJB2 gene, usually causes prelingual, bilateral mild to profound, nonprogressive sensorineural hearing loss. Case presentation: We present an unusual case of an 18-year-old Turkish female with heterozygous 35delG mutation and postlingual, profound-sloping, progressive and fluctuating unilateral sensorineural hearing loss. The phenotype is different from the usual findings. Conclusions: The 35delG mutation causing hearing loss may not always be reflected in the phenotype as expected and therefore may have different audiologic manifestations. [ABSTRACT FROM AUTHOR]

Objective: Vascular disease like small-vessel disease (SVD) is the most likely cause among the potential causes of Sudden sensorineural hearing loss (SSNHL). Understanding the relationship between SVD and SSNHL is crucial for developing effective prevention and treatment strategies. To confirm the relationship between SVD and SSNHL, the effect of SVD is confirmed by focusing on the duration and recurrence of SSNHL. Methods: This article reports a retrospective observational study that investigated the relationship between SVD and SSNHL using the South Korea Health Insurance Review and Assessment Service (HIRA) database from 2010 to 2020. This retrospective observational study included 319,569 SSNHL patients between 2010 and 2020. Results: Participant demographics were controlled using Propensity Score Matching. The hazard ratios (HR) for the effect of SVD on the duration of SSNHL were 1.045 for the group with SVD before the onset of SSNHL and 1.234 for the group with SVD after the onset of SSNHL. SVD was statistically significant for the recurrence of SSNHL, with an odds ratio of 1.312 in the group with SVD compared to the group without SVD. The HR for the period until a recurrence in the group with SVD was 1.062. Conclusions: The study identified SVD as a possible cause of SSNHL and found that the duration of SSNHL increased only in the presence of SVD. SVD also affected the recurrence of SSNHL, with the recurrence rate being 1.312 times higher in the group with SVD. [ABSTRACT FROM AUTHOR]

Background: Petrous apex cholesterol granulomas (PACGs) are benign inflammatory cystic lesions of the temporal bone. Usually, asymptomatic patients may develop symptoms as the lesions expand. The diagnosis is based on both CT and MRI scans and the management relies on "wait and scan" or surgery. This paper aims at evaluating surgical outcomes and proposing a CT-based classification and a management algorithm. Methods: Patients with PACGs who were surgically treated between 2014 and 2024 were included. Symptoms, imaging, approach type and complications were considered. CT scans were classified as Type A (preserved apex cellularity), Type B (erosion of the apex cellularity), and Type C (involvement of the noble structures bone boundaries). The possible connection of the lesion with the infracochlear, perilabyrinthine and sphenoidal cellularity was assessed. Results: Nineteen patients with symptoms like diplopia, headache and sensorineural hearing loss were included. According to our classification, 1/19 patients was Type A, 4/19 were Type B and 14/19 were Type C. Five patients underwent a total resection, seven a subtotal and seven a surgical drainage. Only two complications were recorded, and 17/19 patients showed symptom regression and stability during follow-up. Conclusions: While the management of PACGs is still controversial, according to our classification and surgical outcomes, Type A, being mostly asymptomatic, should be managed with "wait and scan", Type B should undergo surgery when symptoms are present, while Type C should always undergo surgery because of their invasiveness and potential complications. When possible, a drainage should be attempted; otherwise, a surgical resection is chosen, and its completeness depends on the preoperative general and hearing status. [ABSTRACT FROM AUTHOR]

Background: Sildenafil, a selective inhibitor of phosphodiesterase type 5 (PDE5), is widely used for the treatment of erectile dysfunction (ED). However, the safety profile of sildenafil, including adverse event (AEs), requires comprehensive evaluation. Methods: This retrospective pharmacovigilance study aimed to evaluate AEs linked to sildenafil by analyzing data sourced from the FDA Adverse Event Reporting System (FAERS) database. A case/non‐case design was utilized, and various algorithms including the reporting odds ratio (ROR), the proportional reporting ratio (PRR), the Bayesian confidence propagation neural network (BCPNN), and the multiitem gamma Poisson shrinker (MGPS) were employed to measure the signals indicating the presence of sildenafil‐related AEs. Results: Among 339,230 reports, 33,692 specifically mentioned sildenafil use. Most of AEs occurred in males over 60 years old. The United States accounted for the highest proportion of reported AEs. Severe outcomes, including death, disability, and life‐threatening events, were reported. Significant system organ class (SOC) included "Reproductive system and breast disorders" (SOC: 10038604), "Neoplasms benign, malignant and unspecified" (SOC: 10038738), "Vascular disorders" (SOC: 10047065), and "Blood and lymphatic system disorders" (SOC: 10005329). Noteworthy preferred terms (PTs) associated with sildenafil included "Vision blurred," "Flushing," "sudden hearing loss," "Painful erection," and "Priapism." Unexpected AEs, such as "Malignant melanoma," "Pulmonary hypertension," "Malignant melanoma in situ," "Pulmonary arterial hypertension," "Metastatic malignant melanoma," "Malignant melanoma stage III," "Malignant melanoma stage II," "Acquired hemophilia," "Aortic dissection rupture," and "Intracranial artery dissection" were also identified. Conclusions: These findings emphasize the importance of monitoring and understanding the potential risks associated with sildenafil. Further investigation is warranted to validate these associations and address previously unrecognized safety concerns. [ABSTRACT FROM AUTHOR]

Executive functions (EFs) are related abilities, associated with the frontal lobes functions, that allow individuals to modify behavioral patterns when they become unsatisfactory. The aim of this study was to assess EFs in children with sensorineural hearing loss (SNHL) and in children with "specific language impairment" (SLI), compared with a control group of children with normal development, to identify specific skill deficits. Three groups of preschool children aged between 2 and 6 years were assessed: 19 children with normal hearing, cognitive, and language development, 10 children with SNHL, and 20 children with SLI. The FE-PS 2-6 Battery was used for the assessment of preschool EFs, supplemented with the Modified Bell Test for the analysis of selective attention. Statistically significant differences were found between the two experimental groups and the control one, regarding the investigated skills. Children with SNHL showed a clear deficit in flexibility, whereas children with SLI had greater problems in self-regulation and management of waiting for gratification. Selective attention was found to be deficient in all three groups, with no statistically significant differences. This study shows that the skills investigated were found to be deficient in both SNHL and SLI patients. It is essential to start targeted exercises based on specific deficient skills as part of the rehabilitation program. It is of great importance to understand the consequences of EF deficit in preschool children to achieve an accurate diagnosis and carry out customized rehabilitation programs. [ABSTRACT FROM AUTHOR]

Bacckground: Approximately 80% to 85% of sudden sensorineural hearing loss (SSNHL) is idiopathic, but immune-mediated mechanisms are thought to be involved. Behçet disease is an autoinflammatory vasculitis that may involve vessels of the inner ear. It can cause sensorineural hearing loss (SNHL) and an increased risk of SSNHL. Study Sample: We report a 21-year-old man who was diagnosed with Behçet disease in childhood and presented to our clinic with a 10-day history of abrupt hearing deterioration in both ears. Pure-tone audiometry showed severe to profound bilateral SNHL. Results: Oral prednisolone was prescribed for 3 weeks. Concurrent intratympanic steroid injections (ITSIs) were administered in each ear every 2 days for 5 days. A total of 15 daily sessions of hyperbaric oxygen therapy (HBOT) were completed. Acupuncture was performed every 2 days for 1 month. After these combined therapies, the patient's hearing threshold by 20 dB and his speech recognition threshold were improved. Conclusions: Some patients with SSNHL recover no hearing improvement after routine treatment, and alternative treatments including ITSI, HBOT, and acupuncture can be considered as optional. We used an aggressive multimodal approach to treat severe bilateral SSNHL in patient with Behçet disease. [ABSTRACT FROM AUTHOR]

Objective: The etiology of sudden sensorineural hearing loss (SSNHL) remains unknown; inflammation may be one cause. We retrospectively studied SSHNL cases in terms of the inflammatory parameters involved. Methods: We reviewed 169 SSNHL cases. The control group contained 132 patients with vocal cord polyps. The C-reactive protein (CRP) level, CRP/serum albumin (Alb) ratio, and neutrophil/lymphocyte ratio (NLR) were compared between the 2 groups. The relationships between these parameters and the time to treatment after SSHNL onset, hearing level, and therapeutic effects were also analyzed. Results: The CRP level, CRP/Alb ratio, and NLR were higher in SSNHL patients than in controls, but only the NLR differed significantly between the 2 groups. We found no significant differences between patients with different hearing levels, those evaluated at various times after SSNHL onset, and those with different outcomes. Conclusion: The NLR is a reliable SSNHL diagnostic marker. The CRP level and CRP/Alb ratio are not useful indicators. [ABSTRACT FROM AUTHOR]

Hearing loss affects nearly 1.6 billion people and is the third-leading cause of disability worldwide. Chronic kidney disease (CKD) is also a common condition that is associated with adverse clinical outcomes and high health-care costs. From a developmental perspective, the structures responsible for hearing have a common morphogenetic origin with the kidney, and genetic abnormalities that cause familial forms of hearing loss can also lead to kidney disease. On a cellular level, normal kidney and cochlea function both depend on cilial activities at the apical surface, and kidney tubular cells and sensory epithelial cells of the inner ear use similar transport mechanisms to modify luminal fluid. The two organs also share the same collagen IV basement membrane network. Thus, strong developmental and physiological links exist between hearing and kidney function. These theoretical considerations are supported by epidemiological data demonstrating that CKD is associated with a graded and independent excess risk of sensorineural hearing loss. In addition to developmental and physiological links between kidney and cochlear function, hearing loss in patients with CKD may be driven by specific medications or treatments, including haemodialysis. The associations between these two common conditions are not commonly appreciated, yet have important implications for research and clinical practice. Chronic kidney disease is associated with a graded and independent excess risk of sensorineural hearing loss. This Review describes how disruption of shared signalling pathways that are important for the development of both the ear and the kidney and/or the presence of clinical drivers, such as specific medications or treatments, may underlie these associations. Key points: Chronic kidney disease (CKD) and hearing loss are common conditions that individually cause tremendous morbidity but which often coexist. The kidneys and the hearing organs share a common morphogenetic origin and rely on similar biological structures (for example, cilia) and processes (for example, specialized cellular transport mechanisms) to function. Genetic abnormalities that cause CKD can also cause hearing loss, and vice versa. A strong, graded and independent relationship exists between kidney function and the risk of hearing loss; the highest risk is observed in patients on haemodialysis, but kidney transplant recipients and people with mild CKD are also at increased risk. Infants, children and possibly adults with malformation or dysfunction of their hearing organs should be evaluated for the presence of malformation or dysfunction of their kidneys, and vice versa. Additional funding is needed to support basic, clinical and health services research that explores the intersection between these two relatively neglected conditions. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to evaluate the sensory processing abilities of adults with acquired hearing loss and determine whether their sensory processing patterns differ from those of the general population and adults with normal hearing. Method: The study evaluated the sensory processing functions of 30 adults with acquired hearing loss using the Adolescent/Adult Sensory Profile and compared them with the sensory processing functions of 30 adults with normal hearing. Results: The results showed that individuals with hearing loss have a significantly higher sensitivity to stimuli related to motion, vision, activity and touch, exhibiting a low-registration sensory pattern and a sensation-avoiding pattern that differed from those of most individuals. Conclusion: Assessing sensory processing profiles can help identify specific sensory difficulties and inform individualised treatment plans. The study highlights the importance of considering sensory processing patterns in the management of hearing loss to improve overall well-being and quality of life for adults with hearing loss. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the mental health of paediatric cochlear implant users and analyse the relationship between six dimensions (movements, cognitive ability, emotion and will, sociality, living habits and language) and hearing and speech rehabilitation. Methods: Eighty-two cochlear implant users were assessed using the Mental Health Survey Questionnaire. Age at implantation, time of implant use and listening modes were investigated. Categories of Auditory Performance and the Speech Intelligibility Rating Scale were used to score hearing and speech abilities. Results: More recipients scored lower in cognitive ability and language. Age at implantation was statistically significant (p < 0.05) for movements, cognitive ability, emotion and will, and language. The time of implant usage and listening mode indicated statistical significance (p < 0.05) in cognitive ability, sociality and language. Conclusion: Timely attention should be paid to the mental health of paediatric cochlear implant users, and corresponding psychological interventions should be implemented to make personalised rehabilitation plans. [ABSTRACT FROM AUTHOR]

This study aims to determine the prevalence of vestibular schwannoma (VS) among patients presenting with sudden hearing loss (SHL) and to characterize the clinical features of individuals diagnosed with both VS and SHL. We conducted an observational retrospective review at a tertiary referral center, spanning a 30-year period, focusing on patients diagnosed with SHL where VS was confirmed as the underlying cause. We included patients meeting these criteria while excluding those lacking imaging or with a pre-existing diagnosis of VS. We evaluated the audiological characteristics at the time of diagnosis and assessed clinical outcomes following treatment. Among the 403 patients presenting with SHL during the study period, 9 (2.2%) were diagnosed with VS, aged between 25 and 72 years. Although audiometric profiles varied, high-frequency hearing loss predominated, mostly categorized as mild to moderate. Six patients (66%) had Koos grade I-II schwannomas. Only 2 patients achieved complete hearing recovery post-treatment, while 4 showed no improvement. VS is a rare etiology of SHL, accounting for slightly over 2% of cases. Its symptomatology, severity, and audiometric patterns do not significantly differ from SHL caused by other factors. Tumor size does not correlate with hearing characteristics. Treatment modalities resemble those for other SHL cases, and hearing improvement does not obviate the necessity for follow-up magnetic resonance imaging (MRI) scans. [ABSTRACT FROM AUTHOR]

Nervus intermedius (NI) arises from the superior salivary nucleus, solitary nucleus, and trigeminal tract. It leaves the pons as 1 to 5 roots and travels between the facial and vestibulocochlear nerves before merging with the facial nerve within the internal auditory canal. The mastoid segment of the facial nerve then gives rise to a sensory branch that supplies the posteroinferior wall of the external auditory meatus and inferior pina. This complex pathway renders the nerve susceptible to various pathologies, leading to NI neuralgia. Here, the authors present an unusual intraoperative finding of an atrophic NI in a patient with refractory NI neuralgia and a history of ipsilateral sudden-onset central facial palsy and microvascular decompression for trigeminal neuralgia. The patient underwent NI sectioning via the previous retrosigmoid window and achieved partial ear pain improvement. The gross size of the NI is compared with a cadaveric specimen through stepwise dissection. This case highlights the potential significance of subtle central ischemic events and subsequent atrophy of NI in the pathogenesis of NI neuralgia, as well as the ongoing need to investigate the therapeutic efficacy of nerve sectioning. [ABSTRACT FROM AUTHOR]

This study investigated the adjustment behaviour of hearing aid (HA) users participating in a semi-supervised self-adjustment fine-tuning procedure for HAs. The aim was to link behaviour with the reproducibility and duration of the adjustments. Participants used a two-dimensional user interface to identify their HA gain preferences while listening to realistic sound scenes presented in a laboratory environment. The interface allowed participants to adjust amplitude (vertical axis) and spectral slope (horizontal axis) simultaneously. Participants were clustered according to their interaction with the user interface, and their search directions were analysed. Twenty older experienced HA users were invited to participate in this study. We identified four different archetypes of adjustment behaviour (curious, cautious, semi-browsing, and full-on browsing) by analysing the trace points of all measurements for each participant. Furthermore, participants used predominantly horizontal or vertical paths when searching for their preference. Neither the archetype, nor the search directions, nor the participants' technology commitment was predictive of the reproducibility or the adjustment duration. The findings suggest that enforcement of a specific adjustment behaviour or search direction is not necessary to obtain fast, reliable self-adjustments. Furthermore, no strict requirements with respect to technology commitment are necessary. [ABSTRACT FROM AUTHOR]