Severe Hearing Loss Research Articles

Hearing loss prevalence and burden of disease in China: Findings from provincial-level analysis.

Without timely and effective rehabilitation, hearing loss may profoundly affect human life quality. China has a large population of hearing-impaired individuals, which imposes a heavy health burden on society. Moreover, this population is projected to increase rapidly owing to China's aging society. We used data from a population-representative epidemiological investigation of hearing loss and ear diseases in four Chinese provinces. We estimated the national prevalence using multiple linear regression of the age-group proportions and prevalence in 31 provinces with clustering analysis. We used years lived with disability (YLDs) to analyze the disease burden and forecasted the prevalence of hearing loss by 2060 in China. An estimated 115 million people had moderate-to-complete hearing loss in 2015 across the 31 provinces of China (8.4% of 1.37 billion people). Of these, 85.7% were older than age 50years (99 million people) and 2.4% were younger than 20years old (2.8 million people). Of all YLDs attributable to hearing loss, 68.9% were attributable to moderate-to-complete cases. By 2060, a projected 242 million people in China will have moderate-to-complete hearing loss, a 110.0% increase from 2015. The hearing loss prevalence in China is high. Population aging and socioeconomic factors substantially affect the prevalence and severity of hearing loss and the disease burden. The prevalence and severity of hearing loss are unevenly distributed across different provinces. Future public health policies should take these trends and regional variations into account.

Full Recovery of Hearing After Resection of a Large Epidermoid Tumor at the Cerebellopontine Angle: 2-Dimensional Operative Video.

Epidermoid tumors are benign, slow-growing lesions, originating from misplaced ectodermal cells that become trapped during neural tube closure.1 The cerebellopontine angle (CPA) is the most common intracranial location for epidermoid tumors, accounting for approximately 60% of cases.2 Treatment of epidermoid tumors consists of surgical resection, with the goal of gross total resection.3 Here, we describe the case of a patient with a large epidermoid tumor at the CPA causing near-complete hearing loss, who remarkably experienced full recovery of hearing after resection of the tumor. The patient is a 37-year-old woman who presented to our clinic with a CPA tumor causing severe hearing loss consisting of class D hearing and a word recognition score of 5% on audiological examination. Radiographically, the tumor demonstrated significant mass effect on the right cranial nerves VII and VIII with prominent extension into the internal auditory canal. Given the patient's profound hearing loss, she consented to receive a right retrosigmoid craniotomy for resection of the lesion. Although cranial nerves VII and VIII were heavily invested in the tumor, we were able to systematically resect the lesion from the CPA and internal auditory canal, and achieve a gross total resection. Histological examination confirmed the diagnosis of an epidermoid tumor. Remarkably, the patient's audiogram at 3-month follow-up demonstrated complete recovery of hearing in her right ear with a word recognition score of 100% and normal hearing sensitivity across all tested frequencies.

Study on the correlation of C-reactive protein/albumin ratio with sudden sensorineural hearing loss complicated by hypertension: a prospective study.

Understanding the pathophysiology of sudden sensorineural hearing loss (SSNHL) and identifying its clinical symptoms and associated risk factors are crucial for doctors in order to create effective prevention and therapeutic methods for this prevalent otolaryngologic emergency. This study focuses on investigating the correlation between the C-reactive protein/albumin ratio (CAR) and SSNHL complicated by hypertension. In this study, 120 patients diagnosed with SSNHL were divided into groups with and without hypertension, and propensity score matching was used to compare and analyze the severity, type, prognosis, and CAR levels in SSNHL. The results showed that the SSNHL group with hypertension had significantly higher CAR levels, age, hearing curve abnormalities, and more severe hearing loss compared to the control group with isolated SSNHL. These differences were statistically significant (p < 0.001). Among different subtypes of SSNHL, CAR levels increased progressively with the advancement of the condition, and these differences were also statistically significant (p < 0.001). In summary, in patients with SSNHL, those with hypertension had higher CAR levels than those without a history of hypertension, and they experienced more severe hearing loss. Moreover, there was a clear correlation between CAR levels and the extent of SSNHL, indicating that greater CAR levels in patients with SSNHL are connected to more severe hearing loss in various hearing patterns and perhaps indicative of a poorer prognosis.

Cesarean birth and adverse birth outcomes among sub-populations of deaf and hard-of-hearing people

BackgroundDeaf and hard-of-hearing (DHH) people are at higher risk than their non-DHH counterparts of experiencing adverse birth outcomes. There is a lack of research focusing on social, linguistic, and medical factors related to being DHH which may identify groups of DHH people who experience more inequity. ObjectiveExamine difference in prevalence of cesarean and adverse birth outcomes among diverse sub-groups of DHH people. MethodsWe conducted a cross-sectional survey of DHH birthing people in the U.S. who gave birth within the past 10 years. The sample was predominantly white, college educated, and married. We assessed cesarean birth and three adverse birth outcomes: preterm birth, low birthweight, and NICU admission post-delivery. DHH-specific variables were genetic etiology of hearing loss, preferred language (i.e., American Sign Language, English, or bilingual), severity of hearing loss, age of onset of hearing loss, and self-reported quality of perinatal care communication. We estimated prevalence, 95 % confidence intervals, and unadjusted prevalence ratios. ResultsThirty-one percent of our sample reported a cesarean birth. Overall, there were no significant differences in prevalence across the outcome variables with respect to preferred language, genetic etiology, severity, and age of onset. Poorer perinatal care communication quality was associated with higher prevalence of preterm birth (PR = 2.37) and NICU admission (PR = 1.91). ConclusionsOur study found no evidence supporting differences in obstetric outcomes among DHH birthing people across medical factors related to deafness. Findings support the important role of communication access for DHH people in healthcare environments.

“A lonely time for deaf and hard of hearing kids”: A qualitative study of the impact of pandemic precautions on classroom communication for adolescents with hearing loss

BackgroundPandemic public health measures, such as masks and social distancing, present unique challenges for people who are hard-of-hearing. This study sought to understand how adolescents with varying levels of hearing loss would describe their experiences communicating in a classroom environment during a pandemic and its associated public health measures. MethodsQualitative study utilizing one-on-one semi-structured interviews conducted from July 2021 to April 2022. Interviews were transcribed and analyzed using a reflexive thematic analysis to conceptualize the main themes from the data. Adolescents ages 12–17 who attended school in person during the COVID-19 pandemic with either normal hearing or; bilateral cochlear implants or; bone-anchored hearing aids or; unilateral moderate to severe (40–70 dB) conductive hearing loss secondary to mastoidectomy, were interviewed. Participants were recruited from BC Children's Hospital Otolaryngology clinic via a convenience sample. ResultsFourteen adolescents were interviewed with a median age of 15, 9 with hearing loss and 5 with normal hearing. Pandemic associated challenges such as masks muffling speech, protocol fatigue, and missing pre-pandemic life were present in both the hearing loss and normal hearing groups. Classroom communication for adolescents with hearing loss was disproportionately affected by pandemic measures, leading to challenges making friends, feeling behind their peers in learning, and listening fatigue. Resilience was noted among adolescents with hearing loss in their ability to adapt to pandemic measures and changing classroom dynamics. For adolescents with unilateral hearing loss, the pandemic provided an improved listening environment via a reduction in background noise. ConclusionPre-existing classroom communication challenges for adolescents with hearing loss were amplified under pandemic public health measures and shared, in part, by those with normal hearing. These findings can be used to further inform classroom design to the optimize learning environment for deaf and hard of hearing students.

Analysis of clinical manifestations and imaging features of facial nerve schwannomas

Objective:To explore the clinical manifestations and imaging characteristics, and to clarify the imaging value in the diagnosis of facial nerve schwannomas. Methods:Retrospectively analyze the data of 23 patients with facial nerve schwannomas confirmed by surgery and pathology in the Department of Otorhinolaryngology of the First Affiliated Hospital of the Air Force Military Medical University from September 2020 to September 2022, including 8 males and 15 females, aged 18-66 years old. Summarize and analyze their clinical symptoms, specialized examinations, and imaging findings. Results:The clinical manifestations were facial nerve paralysis in 15 cases（2 cases of HB Ⅳ, 6 cases of HB Ⅴ, 7 cases of HB Ⅵ）, hearing loss in 14 cases（5 cases of conductive deafness, 2 cases of mixed deafness, and 7 cases of severe sensorineural hearing loss）, 8 cases tinnitus, 7 cases ear pain, 4 cases dizziness, 4 cases headache, 2 cases ear pus, and parotid gland tumors in 6 cases presenting as local masses. Endoscopic examination revealed 8 cases of external ear canal tumors and 3 cases of intratympanic tumors. Combining temporal bone HRCT, MRI enhanced scanning, and CPR imaging techniques, 1 case involved the internal auditory canal segment, 2 cases in the tympanic segment, 6 cases in the parotid gland area. A total of 14 cases involved two or more segments of the internal auditory canal segment, the labyrinthine segment, geniculate ganglion, the tympanic segment, and the mastoid segment. When the tumors were large, adjacent structures were involved. It was found that 8 cases invaded the external auditory canal and tympanic cavity, ossicles were displaced or bony destruction; 3 cases invaded the jugular foramen area, and 1 case grew to the middle cranial fossa region with temporal lobe brain parenchymal compression. Conclusion:The clinical manifestations of facial nerve schwannomas are diverse. The combination of various imaging techniques will be conducive to topical and qualitative diagnosis and provide an important basis for treatment strategies.

Audiological Profile in Adenoid Hypertrophy.

The study aims to investigate the correlation between grade of adenoid hypertrophy and severity of hearing loss and to profile tympanometric findings in children with Adenoid Hypertrophy. A within group comparison study was carried out in Father Muller College, Department of Speech and Hearing, Mangalore where 123 patients diagnosed with Adenoid Hypertrophy (86 males and 37 females; mean age 7.146; range 2 to 12 years) were analyzed using detailed case history, Pure tone audiometry (PTA) and Tympanometry. The most prevalent presenting problems with adenoid hypertrophy were snoring (68.29%), mouth breathing (57.72%)and reduced hearing (35.77%). PTA results showed majority had bilateral hearing loss (60.27%). In the 230 ears that were tested, 50.85% had hearing loss. Majority of these ears had a minimal loss (23.91%), followed by mild loss (18.69%), moderate loss (7.82%), and moderately severe loss (0.43%). No correlation was found between the grade of Adenoid hypertrophy and the severity of hearing loss noted (p > 0.05). According to tympanometric findings, the most common tympanogram pattern was 'B' type (39.15%), 'A' type (31.60%), and 'C' type (18.39%). A small proportion of the population had 'As' (4.71%), 'Cs' (5.66%), and 'Ad' (0.47%).No correlation was found between the grade of adenoid hypertrophy and the type of tympanogram obtained (p > 0.05). In a significant percentage of cases, Adenoid hypertrophy affects the middle ear leading to conductive hearing loss. If left untreated it can lead to delayed speech and language development, auditory processing disorders, mental retardation, and physical and social complications. These are avoidable through primary health care education, accurate diagnosis, and effective treatment. The online version contains supplementary material available at 10.1007/s12070-024-04714-8.

Neurobehavioral outcomes of neonatal asymptomatic congenital cytomegalovirus infection at 12-months

BackgroundCongenital cytomegalovirus (cCMV) is the most common congenital viral infection in the United States. Symptomatic infections can cause severe hearing loss and neurological disability, although ~ 90% of cCMV infections are asymptomatic at birth. Despite its prevalence, the long-term neurobehavioral risks of asymptomatic cCMV infections are not fully understood. The objective of this work was to evaluate for potential long-term neurobehavioral sequelae in infants with asymptomatic cCMV.MethodsInfants with cCMV were identified from a universal newborn cCMV screening study in a metropolitan area in the midwestern United States. Asymptomatic infants with cCMV were enrolled in a longitudinal neurodevelopmental study (N = 29). Age- and sex-matched healthy control infants (N = 193) were identified from the Baby Connectome Project (BCP), a longitudinal study of brain and behavioral development. The BCP sample supplemented an additional group of healthy control infants (N = 30), recruited from the same participant registry as the BCP specifically for comparison with infants with asymptomatic cCMV. Neurobehavioral assessments and parent questionnaires, including the Mullen Scales of Early Learning, the Repetitive Behavior Scales for Early Childhood (RBS-EC), and the Infant Toddler Social Emotional Assessment (ITSEA) were administered at 12 months of age. Neurobehavioral scores were compared between infants with asymptomatic cCMV and all identified healthy control infants.ResultsInfants with asymptomatic cCMV performed equivalently compared to healthy control infants on the neurobehavioral measures tested at 12 months of age.ConclusionsThese results indicate that at 12 months of age, infants with asymptomatic cCMV are not statistically different from controls in a number of neurobehavioral domains. Although follow-up is ongoing, these observations provide reassurance about neurobehavioral outcomes for infants with asymptomatic cCMV and inform the ongoing discussion around universal screening. Additional follow-up will be necessary to understand the longer-term outcomes of these children.

To Study the Pre Operative Indicators of Incudal Necrosis in Tubo-Tympanic Chronic Otitis Media.

To assess the pre-operative indicators of incudal necrosis in tubo-tympanic CSOM. A total of 80 patients with tubo-tympanic type of CSOM attending the SMGS Hospital ENT OPD/IPD of both genders were enrolled for the study. Pre-operative findings on oto-microscopic examination, pure-tone audiometry, x-ray mastoid and intra-operative microscopy was recorded. It was found that patients with age > 30years and more than 10years of CSOM had had higher incidence of ossicular necrosis as compared to patients age < 30years and less than 10years of CSOM (P < 0.05). Patients who had granulation tissue and moderate to moderately severe hearing loss had higher incidence of ossicular necrosis as compared to other patients. The difference was seen significant (P < 0.05). In this study, it was observed that the presence of granulations over the perforation margins, disease persisting for more than 10years, moderate to moderately severe hearing loss appear to be significant reliable indicators of incudal necrosis in tubo-tympanic type of CSOM. All patients of tubo-tympanic type of CSOM should be assessed in detail so as to reach early diagnosis of ossicular erosion that helps in surgical decision making and preparedness regarding ossiculoplasty and patient consent.

The Impact of Cardiovascular Risk Factors on the Incidence, Severity, and Prognosis of Sudden Sensorineural Hearing Loss (SSHL): A Systematic Review.

Sudden sensorineural hearing loss (SSHL) is believed to be mainly idiopathic since the cause is not usually identified. Several recent studies have examined the role of cardiovascular risk factors in this disease.The aim of this systematic literature review is to investigate the possible association between acquired and inherited cardiovascular risk factors and the incidence, severity, and prognosis of SSHL. A systematic review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A search of the PubMed database for the period between February 2010 and January 2023 was performed in order to retrieve eligible articles. The analytic cohort included 24 studies. Overall, this systematic review includes a total of 61,060 patients that were encompassed in these studies. According to most studies, the prevalence of dyslipidaemia, diabetes, and ultrasound indices of atherosclerosis was significantly higher in SSHL patients compared to controls. On the other hand, obesity, hypertension, and smoking did not seem to influence the risk of SSHL. Most studies suggest the presence of a correlation between a high cardiovascular risk profile and the risk of developing SSHL. The theory of microvascular impairment in the development of SSHL is indirectly supported by the findings of this review.

Novel likely pathogenic variant in the EYA1 gene causing Branchio oto renal syndrome and the exploration of pathogenic mechanisms

ObjectiveBranchio-oto-renal syndrome (BOR, OMIM#113,650) is a rare autosomal dominant disorder that presents with a variety of symptoms, including hearing loss (sensorineural, conductive, or mixed), structural abnormalities affecting the outer, middle, and inner ear, branchial fistulas or cysts, as well as renal abnormalities.This study aims to identify the pathogenic variants by performing genetic testing on a family with Branchio-oto-renal /Branchio-otic (BO, OMIM#602,588) syndrome using whole-exome sequencing, and to explore possible pathogenic mechanisms.MethodsThe family spans 4 generations and consists of 9 individuals, including 4 affected by the BOR/BO syndrome. Phenotypic information, including ear malformation and branchial cleft, was collected from family members. Audiological, temporal bone imaging, and renal ultrasound examinations were also performed. Whole-exome sequencing was conducted to identify candidate pathogenic variants and explore the underlying molecular etiology of BOR/BO syndrome by minigene experiments.ResultsIntra-familial variability was observed in the clinical phenotypes of BOR/BO syndrome in this family. The severity and nature of hearing loss varied in family members, with mixed or sensorineural hearing loss. The proband, in particular, had profound sensorineural hearing loss on the left and moderate conductive hearing loss on the right. Additionally, the proband exhibited developmental delay, and her mother experienced renal failure during pregnancy and terminated the pregnancy prematurely. Genetic testing revealed a novel heterozygous variant NM_000503.6: c.639 + 3 A > C in the EYA1 gene in affected family members. In vitro minigene experiments demonstrated its effect on splicing. According to the American College of Medical Genetics (ACMG) guidelines, this variant was classified as likely pathogenic.ConclusionThis study highlights the phenotypic heterogeneity within the same family, reports the occurrence of renal failure and adverse pregnancy outcomes in a female patient at reproductive age with BOR syndrome, and enriches the mutational spectrum of pathogenic variants in the EYA1 gene.

Detailed Clinical Features of PTPRQ-Associated Hearing Loss Identified in a Large Japanese Hearing Loss Cohort.

The PTPRQ gene has been identified as one of the genes responsible for non-syndromic sensorineural hearing loss (SNHL), and assigned as DFNA73 and DFNB84. To date, about 30 causative PTPRQ variants have been reported to cause SNHL. However, the detailed clinical features of PTPRQ-associated hearing loss (HL) remain unclear. In this study, 15,684 patients with SNHL were enrolled and genetic analysis was performed using massively parallel DNA sequencing (MPS) for 63 target deafness genes. We identified 17 possibly disease-causing PTPRQ variants in 13 Japanese patients, with 15 of the 17 variants regarded as novel. The majority of variants identified in this study were loss of function. Patients with PTPRQ-associated HL mostly showed congenital or childhood onset. Their hearing levels at high frequency deteriorated earlier than that at low frequency. The severity of HL progressed from moderate to severe or profound HL. Five patients with profound or severe HL received cochlear implantation, and the postoperative sound field threshold levels and discrimination scores were favorable. These findings will contribute to a greater understanding of the clinical features of PTPRQ-associated HL and may be relevant in clinical practice.

Geographic, Sociodemographic, and Clinical Factors Associated With Parental Self-Efficacy in Pediatric Patients With Hearing Loss.

To identify geographic, sociodemographic, and clinical factors associated with parental self-efficacy in a diverse cohort of deaf or hard-of-hearing (DHH) children. Cross-sectional study. Tertiary children's hospital. Four hundred forty parents of DHH children aged 0 to 17 completed the 25-item Scale of Parental Involvement and Self-Efficacy (SPISE) survey from 2014 to 2022. Residential addresses were geocoded and assigned Area Deprivation Index and Social Vulnerability Index rankings, and univariable and multivariable analyses were conducted using sociodemographic and clinical variables, including sex, race/ethnicity, insurance type, survey language, age at the survey, comorbidities, newborn hearing screening results, and hearing loss laterality and severity. Compared to English and Spanish-speaking parents, Chinese-speaking parents were associated with overall lower parental self-efficacy and involvement (regression coefficient = -0.518, [-0.929, -0.106]), Cohen's d = 0.606) and lower scores on items related to their ability to affect multiple aspects of their child's development and expression of thoughts as well as competency in checking and putting on their child's sensory device. Across univariable and multivariable analyses, besides Chinese language, all other sociodemographic, clinical, and geographic variables were not associated with SPISE score. To achieve the best patient outcomes, care teams can use the SPISE to evaluate parental self-efficacy and provide targeted support to parents at risk for having lower knowledge and confidence scores about critical skills necessary to facilitate their child's auditory access and language development. Notably, this study found similar reports of parental efficacy across various sociodemographic, clinical, and geographic variables but significantly lower SPISE scores in Chinese-speaking families.

Prevalence of hearing loss in patients with sickle cell disease in the southeast of Iran

Background: One of the complications of sickle cell disease (SCD) is hearing loss. The purpose of the present study is to determine the prevalence of hearing loss in SCD.&#x0D; Materials and Methods: This is a descriptive-analytical cross-sectional study conducted on 100 patients with SCD in 2019-2020. All the patients underwent otololaryngological examination and audiometric tests to assess their hearing status. The type and severity of hearing loss were determined, the demographic information of the patients was recorded, and the data were analyzed using the SPSS software version 21, a Chi-square test, and the Kruskal-Wallis test. The P-values of &lt; 0.05 were considered statistically significant.&#x0D; Results: The mean age of the patients was 17.6 ± 10.8 years. Of them, 60% were male. Hearing loss was diagnosed in 26 patients (26%), 16 of whom had sensorineural hearing loss (SNHL) and 10 had conductive hearing loss (CHL). In terms of the disease severity, 12, 10, 3 and 1 patients had mild, moderate, severe and profound hearing loss, respectively. The mean age of the patients with SNHL was significantly higher than the median age of the subjects with CHL (p-value = 0.043). Also, the patients with hearing loss had no statistically significant difference in terms of the variables of age, gender, unilateral or bilateral ear involvement and type of SCD (p-value = 0.069).&#x0D; Conclusion: This study revealed a hearing loss frequency of 26% among patients with SCD. Regular hearing assessment and timely treatment and rehabilitation measures are recommended for these patients.

Personalized prediction of speech intelligibility for hearing-impaired listeners using a physiological model of the human ear

Due to the problems of occlusion effect, insufficient high-frequency gain, and acoustic feedback in conventional hearing aids, middle ear implants as a new type of hearing device have become an important compensatory tool for the treatment of patients with moderate to severe hearing loss. However, current speech intelligibility (SI) models are inadequate for predicting SI in hearing-impaired (HI) listeners after middle ear implants implantation. Moreover, the compensatory performance of middle ear implants before implantation remains unknown due to the invasive nature of the surgical procedure. Therefore, this study proposes a novel SI model that can predict the compensatory effects on SI after middle ear implants implantation. The model combines a physiologically nonlinear auditory preprocessing front-end with a short-term correlation analysis back-end. In normal-hearing (NH) listeners, the model accurately predicts speech reception thresholds (SRTs) and masking release under steady-state and fluctuating noise conditions. For HI listeners, the model modifies the parameters of outer hair cells and inner hair cells in the preprocessing front-end to simulate the patient’s audiogram, achieving excellent predictive capability for HI listeners’ test data. Overall, the proposed SI model can be used for the optimal design and algorithm fitting of middle ear implants tailored to patients with varying degrees of hearing loss, offering valuable insights for the clinical treatment of HI listeners.

Correlation between Clinical and Audiological Factors with Ossicular Chain Continuity in Chronic Otitis Media

Introduction: Chronic otitis media (COM) with the perforation of the tympanic membrane, myringoplasty is preferred treatment, but if ossicles are involved, myringoplasty is done with ossiculoplasty which is termed as tympanoplasty. Preoperative assessment of ossicular status is important to decide the type of tympanoplasty. Ossicular chain continuity is generally seen in the patient who has short duration of disease and smaller air–bone gap. Our study was conducted to support the relationship between preoperative clinical, audiological, and other factors in relation to the ossicular chain status. Materials and Methods: This was a prospective, cross-sectional study conducted at a tertiary care center, for 2-year duration (November 2019 to November 2021), on 100 patients who presented with COM (mucosal type). Preoperative diagnosis and assessment based on the clinical examination and investigations were compared with operative findings. Results: Thirty percent of the patients had ear discharge duration &lt;1 year, 28% had 1–5 years, and 18% patients had 6–10-year duration. Hearing loss was present in 80% of the cases. Correlation of pure-tone average (PTA) with ossicular chain status was significant with P = 0.028. The majority of patients were in the moderate category 31% and 19.36% of patients had ossicular chain defect. The second common category was mild hearing loss (24%), of which 4.17% of patients showed ossicular chain defect. Conclusion: Maximum percentages of patients with severe hearing loss had ossicular chain discontinuity followed by profound hearing loss. Most of cases had moderate to moderately severe conductive hearing loss in PTA. As the air–bone gap increases, the possibility of ossicular discontinuity increases.

Clinical features of congenitally enlarged bony portion of Eustachian tube

Objective:To investigate the clinical features of patients with congenitally enlarged bony portions of the Eustachian tube（ET）. Methods:The medical history, physical examination, hearing test, temporal bone high resolution computed tomography（HRCT） of six patients（nine ears） with congenitally enlarged bony portion of the ET were retrospectively analyzed. Results:Four patients were men and two were women. The minimum, maximum, and average ages were 5, 21, and（14.7±6.4） years, respectively. Three malformations were bilateral and three were left-sided. Three ears had conductive hearing loss（average bone and air conduction thresholds were 13.7 dB and 71.3 dB）, three had mixed hearing loss（average bone and air conduction thresholds were 27.7 dB and 83.7 dB）, and one had extremely severe sensorineural hearing loss. The average maximum length and width of the enlarged bony ET on temporal bone HRCT were（22.61±2.94） mm and（6.50±2.33） mm, respectively. The enlargement was combined with an external auditory canal malformation in six ears, narrow tympanic cavity in six, tympanic antrum malformation in five, ossicular chain malformation in seven, cochlear malformation in six, helicotrema malformation in three, vestibule widening in two, semicircular canal malformation in three, vestibular window malformation in six, facial nerve abnormality in five, internal auditory meatus malformation in two, low middle cranial fossa in eight, and severe internal carotid artery malformation in one. Conclusion:Bony ET enlargement is a rare congenital middle ear malformation which could combined with other ear malformations. Patients can have no ET dysfunction but different patterns of hearing loss. The defect is usually found unintentionally during imaging, and the HRCT of temporal bone is significant.

Adverse events associated with Vibrant Soundbridge: A MAUDE study

ObjectiveTo summarize adverse events and their root causes reported to the United States Food and Drug Administration (FDA) on Vibrant Soundbridge (VSB) hearing device (Med-El, Innsbruck, Austria), an active middle ear implant for patients with moderate to severe hearing loss. Materials and methodsThe FDA's Manufacturer and User Facility Device Experience (MAUDE) database was queried for reports of VSB adverse events from January 1, 2012, to July 27, 2022. ResultsSix hundred sixty-three total medical device reports were identified, from which 913 adverse events were extracted. Of these, 498 (54.5 %) were adverse events to patients (AEPs), while 415 (45.5 %) were device malfunctions (DMs). The most common AEPs were hearing performance issues 428 (85.9 %). The most common DMs were compromised conductive link 125 (30.1 %). Root causes identified for DMs were iatrogenic 85 (58.6 %), patient-related 28 (19.3 %), and trauma and external causes 32 (22.1 %). The most common iatrogenic root cause 12 (14.1 %) involved damage to the conductive link during revision surgery. The most common patient-related causes of DMs were excessive middle ear tissue growth 16 (57 %), and abrupt body movements 5 (28.6 %). The most common external cause of DM was cleaning of the ear canal or mastoid cavity 20 (62.5 %). ConclusionsDespite its well-known limitations, the MAUDE database provides valuable information on possible complications of VSB as it relates to device malfunction or adverse events for patients. Implementation of standardized reports with relevant and well-defined categories could certainly allow for a more meaningful analysis.

Hearing and Language Skills in Children Using Hearing Aids: Experimental Intervention Study.

Hearing loss in childhood compromises a child's auditory, linguistic, and social skill development. Stimulation and early intervention through therapy and the use of personal sound amplification devices (PSAPs) are important for improving communication. To verify the effectiveness of speech therapy intervention on the auditory and linguistic skills of Brazilian children aged between 6 and 8 years using PSAPs. Experimental study analyzing the intervention process in children aged between 6 and 8 years with mild to severe bilateral hearing loss and prelingual deafness who are PSAP users. Diagnostic information was analyzed, and assessments and interventions were carried out using the Glendonald Auditory Screening Procedure (GASP), a phoneme discrimination test with figures (TFDF), an expressive language category classification test, and an Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) questionnaire. Sixteen children participated in the study; they were divided into a control group (CG) of six children and an intervention group (IG) of ten children. All research subjects underwent two protocol application sessions, and the IG underwent six speech therapy intervention sessions. In the IT-MAIS, the CG had a 9% increase in score, and the IG had an increase of 3% after intervention. The TFDF obtained a 5% increase in the IG in terms of phonemic discrimination ability. The expressive language category classification tests and GASP were considered not sensitive enough to modify the parameters of auditory and linguistic skills. The study found a significant improvement amongst the IG in the TFDF protocol and an increase in IT-MAIS scores in both groups.

Active TriCROS: A Simultaneous Stimulation With a (Bi)CROS System and a Hearing Aid in the Worst Ear for Severely Asymmetrical Hearing Loss.

Severely asymmetrical hearing loss (SAHL) is characterized by a moderately severe or severe hearing loss in one side and normal or mildly impaired controlateral hearing in the other. The Active tri-CROS combines the Contralateral Routing-of-Signal System (CROS, or BiCROS if the best ear is stimulated) and the stimulation of the worst ear by an in-the-canal hearing aid. This study aims to evaluate the benefit of the Active tri-CROS for SAHL patients. This retrospective study was conducted from September 2019 to December 2020. Ambulatory, tertiary care. Patients were retrospectively included if they had received the Active tri-CROS system after having used a CROS or BiCROS system for SAHL for at least 3 years. Audiometric gain, signal-to-noise ratio, spatial localization, and the Abbreviated Profile of Hearing Aid Benefit and Tinnitus Handicap Inventory questionnaires were performed before equipment and after a month with the system. Twenty patients (mean, 62 yr old) with a mean of 74.3 ± 8.7 dB HL on the worst ear were included. The mean tonal hearing gain on the worst ear was 20 ± 6 dB. Signal-to-noise ratio significantly rose from 1.43 ± 3.9 to 0.16 ± 3.4 dB ( p = 0.0001). Spatial localization was not significantly improved. The mean Tinnitus Handicap Inventory test score of the eight patients suffering from tinnitus rose from 45.5 ± 18.5 to 31 ± 25.2 ( p = 0.016). The Active tri-CROS system is a promising new therapeutically solution for SAHL.