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Background: Superior semicircular canal dehiscence (SSCD) is a vestibular-cochlear disorder in humans in which a pathological third mobile window of the otic capsule creates changes to the flow of sound pressure energy through the perilymph/endolymph. The primary symptoms include sound-induced dizziness/vertigo, inner ear conductive hearing loss, autophony, headaches, and visual problems. We have developed an animal model of this human condition in the Mongolian Gerbil that uses surgically created SSCD to induce the condition. A feature that is unique in this model is that spontaneous resurfacing of the dehiscence occurs via osteoneogenesis without a subsequent intervention. In this study, we completed our assessment of this model to include reversible asymmetrical vestibular impairments that interfere with balance. Methods: Adult Mongolian gerbils (N = 6) were trained to complete a balance beam task. They were also trained to perform a Rotarod task. After 10 days of training, preoperative ABR and c+VEMP testing was followed by a surgical fenestration of the left superior semicircular canal. Balance beam testing recommenced at postoperative day 6 and continued through postoperative day 15 at which point final ABR and c+VEMP testing was carried out. Results: Behavioral comparison of preoperative and postoperative performance show a significant decrease in Rotarod performance, increased rates of falling, and an increase in time to cross the balance beam. Impairments were the most significant at postoperative day 7 with a return toward preoperative performance by postoperative day 14. This behavioral impairment was correlated with residual impairments to auditory thresholds and vestibular myogenic amplitudes at postoperative day 14. Conclusion: These results confirm that aberrant asymmetric vestibular output in our model of SSCD results in reversible balance impairments. The level of these behavioral impairments is directly correlated with severity of the vestibular dysfunction as we have previously reported for peripheral ear physiology and cognition. [ABSTRACT FROM AUTHOR]

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

Dural venous sinus stenting is an emerging and exciting area in otolaryngology in collaboration with neurosurgeons and neuroradiologists. The first cases were reported 20 years ago. It is now considered part of the routine treatment of increased intracranial pressure due to transverse sinus stenosis. ENT doctors are the first to see these patients in their clinics, as sinus headaches, pulsating tinnitus, and dizziness are the most common symptoms. Previously, with limited success, high-dose diuretics and intracranial shunts had been the only options for treating these patients. Other methods, such as covering the sigmoid sinuses with graft material, appear to cause a sudden increase in intracranial pressure that can lead to blindness and even death. This overview summarizes the clinical and imaging characteristics of patients who will benefit from endovascular sinus stenting for elevated intracranial pressure. [ABSTRACT FROM AUTHOR]

Objective: A new, active transcutaneous bone conduction device (BCD) was FDA-approved in 2019 in the USA. This systematic review sought to evaluate early outcomes associated with Osia implantation. Methods: A systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines. Four databases were reviewed: PubMed, Scopus, Cochrane CENTRAL, and CINAHL. Studies were included if they described audiometric, surgical characteristics/complications, or adverse events associated with the Osia BCD. Exclusion criteria: non-English language studies, animal investigations, reviews/meta-analyses, case reports, database studies. Results: Eighteen studies with 336 patients were included. Mean age at implantation was 37.9 years. About 79.5% of patients had MHL/CHL and 19.5% had SSD/SHL. Mean operative time was 71.6 minutes. Mean PTA gain from unaided conditions was 35.4 dB. Mean functional gain at high frequency (6 kHz and above) from aided conditions was 16.1 dB. Mean improvement in speech recognition thresholds was 19.1 dB from unaided conditions. Adverse events (all types) were reported in 20.1% of cases. Across all studies, the postoperative infection rate was 5%. About 2% of patients reported magnet retention issues. About 1.65% of cases were complicated by hematomas. Conclusions: Under systematic literature review, the Osia BCD has been associated with low complication rates, relatively short operative times, and good audiometric and speech outcomes, notably high frequency gain >6 kHz. More advanced audiometric outcome reporting remains limited and audiometric data and patient reported outcome measures were reported heterogeneously. [ABSTRACT FROM AUTHOR]

Background Sudden sensorineural hearing loss (SSNHL) is an otologic emergency requiring urgent medical attention and care. Objective This article, based on up-to-date evidence and clinical guidelines, aims to equip general practitioner (GP) specialists with a structured and practical approach to SSNHL management, emphasising the need for prompt evaluation and appropriate interventions. From initial evaluation to treatment strategies and follow-up, this guide offers a step-by-step framework to optimise patient care and improve outcomes in patients suffering SSNHL. Discussion Distinguishing SSNHL from conductive hearing loss (CHL), with early identification and prompt initiation of high-dose corticosteroid therapy, are key considerations for the management of SSNHL. Appropriate referrals when indicated for audiometric evaluation, emergency and/or otolaryngology services are also essential. Furthermore, this study aims to outline emerging therapies including intratympanic steroid administration, hyperbaric oxygen therapy and their potential roles in augmenting standard treatment approaches. [ABSTRACT FROM AUTHOR]

Purpose: Epidemiological studies have demonstrated a relationship between general intelligence (GI) in youth and hearing loss (HL). However, no large-scale study assessed the relations of GI in late adolescence with conductive HL (CHL) and sensorineural HL (SNHL), stratified by severity. This study examined the connection between HL and GI in late adolescence. Methods: Cross-sectional study on mandatory premilitary recruitment data recorded during 1967–2019 of patients aged 17–19. We compared GI between SNHL, CHL, and those with normal hearing. In addition, we used logistic regression to measure the associations between HL and GI after adjuring for age, sex, education, and socioeconomic status. Results: Among 3,104,851 adolescents assessed, 20,075 (0.6%) exhibited HL. We categorized GI into three levels for analysis: low (lowest category), medium, and high (reference category). Adjusted odds ratios (ORs) revealed that SNHL was associated with lower GI levels, with ORs ranging from 1.3 (95% confidence interval [CI] 1.4–1.2) for the lowest GI category to 1.1 (95% CI 1.15–0.04) for the medium category. CHL (CHL) also demonstrated significant associations, with ORs from 1.8 (95% CI 1.9–1.6) for the lowest GI level to 1.1 (95% CI 1.2–0.9) for medium. Further analysis revealed a statistically significant, severity-dependent relationship between SNHL and the odds of being in the lowest GI quartile (Q1). Specifically, the ORs for SNHL ranged from 1.2 (95% CI 1.1–1.3) to 1.3 (95% CI 1.1–1.5) as severity increased, indicating a strong link between greater SNHL severity and reduced cognitive performance. In contrast, CHL did not show a consistent correlation between its severity and GI outcomes, with an OR of 1.6 (95% CI 1.2–2.3) across severity levels. Conclusion: We report a strong relationship between HL and GI in late adolescence. SNHL, but not CHL, demonstrated a severity-based decline in GI. The results highlight the value of early, specifically targeted therapies for HL that consider its etiology and degree. [ABSTRACT FROM AUTHOR]

Background Methods Results Conclusion In Australia, middle ear disease disproportionately affects Indigenous children, leading to poor hearing outcomes. This study aimed to determine the natural history of untreated chronic otitis media in Indigenous children in remote South Australia.Baseline and 3 year follow‐up data was collected from Indigenous children aged 5–18 years living on the Anangu Pitjantjatjara Yankunytjatjara Lands. 4‐frequency pure‐tone audiometry was used to determine hearing levels. Middle ear pathology was determined by video‐otoscopy with tympanometry and classified at baseline as group 1 (normal), group 2 (abnormal with intact tympanic membrane), or group 3 (perforated tympanic membrane).A total of 253 children were included in this study. Children in group 1 (20.6 ± 1.5 dBHL; Mean ± SD) had significantly better hearing outcomes at 3 year follow‐up than children with abnormal ears (groups 2 and 3) (23.8 ± 7.0 dBHL), P < 0.001. The difference was greatest for group 1 versus 3 (27.6 ± 8.4 dBHL), P < 0.001, followed by 1 versus 2 (22.4 ± 5.8 dBHL), P = 0.009, and between 2 (22.4 ± 5.8 dBHL) and 3 (27.6 ± 8.4 dBHL), P < 0.001.Hearing in Indigenous children with untreated middle ear pathology remains poor at follow‐up compared to those without pathology. Intervention is therefore critical to prevent persisting poor hearing outcomes. [ABSTRACT FROM AUTHOR]

Neuroendocrine tumors (NETs) are usually found in the gastrointestinal tract and pancreas; however, they are rare in the middle ear. The presentation of vague symptoms, such as tinnitus and conductive hearing loss, complicates the diagnosis. We present a 46-year-old male with a history of right ear discharge and a previous canal wall-down mastoidectomy, suspected to have cholesteatoma recurrence. Imaging and surgery demonstrated a well-differentiated G1 NET in the middle ear. Postoperative metastatic workup revealed few pulmonary nodules needing ongoing observation. Middle-ear NETs are difficult to diagnose due to their rarity. This case underscores the importance of histological and immunohistochemical evaluation and the need for systemic assessment to rule out metastasis due to pulmonary nodules. We contribute to the limited literature highlighting the need to include NETs in the differential of middle ear masses and the usefulness of interdisciplinary teamwork. [ABSTRACT FROM AUTHOR]

Background: Following primary cleft palate repair, individuals face a heightened risk of hearing problems, particularly conductive hearing loss, compensatory articulation disorders (CADs), resonance disorders, delayed speech and language development, and voice disorders. This study aimed to investigate the prevalence and impact of these challenges in children with cleft palate with or without cleft lip (CP± L). Methods: This cross-sectional study included 38 children with CP± L aged 5 to 13 years. A comprehensive evaluation involved audiological assessments (audiograms, tympanograms) by an audiologist and speech-language pathology assessments (Thai Speech Parameters for Patients with Cleft Palate, Articulation Screening Test) by speech-language pathologists. Results: The prevalence of hearing loss affected 27.63% of participants (21 out of 76 ears) and majority of cases involved conductive hearing loss. Velar substitution was the most common CAD, followed by nasalized voiced pressure consonants, phoneme-specific nasal air emission, and pharyngeal substitution. A moderate correlation was found between these CAD patterns and hypernasality at the word, sentence, and screening levels (r= 0.44, p< 0.01; r= 0.43, p< 0.01; and r= 0.40, p= 0.01). Conclusion: For summary, the most common type of hearing loss was conductive hearing loss. The predominant CAD pattern was velar substitution. The protocol could be designed to enhance early improvement in hearing and articulation, thereby supporting academic achievement and long-term quality of life. [ABSTRACT FROM AUTHOR]

Objectives. This study aimed to compare the audiological benefits of a non-implantable wearable option for a bone conduction (BC) implant mounted on an arch (SoundArc) to those of traditional BC hearing aids (HAs) mounted on eyeglasses (BCHAs) in patients with moderate to severe conductive or mixed hearing loss. Methods: A preliminary cross-sectional observational prospective cohort study was conducted in the Tertiary Audiological Department, University Hospital. Fourteen adults with conductive or mixed hearing loss (PTA at 0.5-1-2-4 KHz = 67 ± 15 dB HL) who had been wearing conventional BCHAs mounted on eyeglasses for at least 3 years and had declined surgical implantation of a bone conduction hearing implant (BCHI) were included in the study. Unaided and aided pure-tone air conduction (AC) and bone conduction (BC) thresholds, as well as speech tests in quiet and noise, were recorded at baseline and in two different settings: with a BCHI mounted on SoundArc® and with their own BCHAs mounted on eyeglasses using two couplers. Participants completed questionnaires in both conditions, including the International Inventory for Hearing Aids (IOI-HA), the Hearing Handicap Inventory for Adults/Elderly (HHIA/E), the Speech, Spatial, and Qualities of Hearing Scale (SSQ), a 10-point visual analog scale (VAS), and the Fatigue Impact Scale (FIS). Results: A significant functional gain was observed in both settings (p = 0.0001). Better speech perception in quiet and noise was observed with SoundArc compared to conventional BCHAs on eyeglasses (improvements in word repetition scores in noise: +19.3 at SNR +10 dB, p = 0.002; +12.1 at SNR 0 dB, p = 0.006; and +11.4 at SNR −10 dB, p = 0.002). No significant differences were found in IOI-HA, FIS, and HHIA/E scores. However, significantly better SSQ scores were reported for SoundArc in all domains (p = 0.0038). Conclusions: Although patients were accustomed to using BCHAs mounted on eyeglasses, the bone conduction wearable option of the BCHI (SoundArc) proved to be a viable alternative for adult patients with conductive or mixed hearing loss who are unable or unwilling to undergo BCHI surgery. [ABSTRACT FROM AUTHOR]

Air and bone conduction thresholds are used to differentiate between conductive and sensori-neural hearing losses because bone conduction is thought to bypass the conductive apparatus, directly activating the inner ear. However, the suggested bone conduction mechanisms involve the outer and middle ears. Also, normal bone conduction thresholds have been reported in cases of lesions to the conduction pathway. Therefore, further investigation of bone conduction mechanisms is required. [ABSTRACT FROM AUTHOR]

Conductive hearing loss caused by external or middle ear problems prevents the transmission of sound waves from the external auditory canal to the cochlea, and it is a common condition, especially in pediatric patients aged 1–5 years. The most common etiological factors are otitis media and cerumen during childhood. In some patients, external and middle ear functions and structures may be normal bilaterally despite the air-bone gap on the audiogram. This condition, which is often a missed diagnosis in children, is defined as a pseudo-conductive hearing loss (PCHL) caused by third window syndromes (TWSs) such as semicircular canal dehiscence, inner ear malformations with third window effect, and perilymphatic fistula. In this review of the literature, the authors emphasize the pitfalls of pediatric audio-vestibular evaluation on TWSs as well as the key aspects of this evaluation for the differential diagnosis of PCHL brought on by TWSs. This literature review will provide audiologists and otologists with early diagnostic guidance for TWSs in pediatric patients. [ABSTRACT FROM AUTHOR]

Objective: The objective of this study is to investigate whether conductive hearing loss (CHL) can be differentiated from sensorineural hearing loss in children ages 3–18 using a diotic and antiphasic digits‐in‐noise (DIN) tablet‐based test using existing adult cut‐off criteria. Methods: A blinded multi‐institutional prospective cohort of 64 children aged 3–18 scheduled for an audiometric soundbooth evaluation with a pediatric audiologist and a same‐day otolaryngologist examination were recruited for the study. Following a conventional audiogram, the subjects underwent diotic (same‐phased stimuli) and antiphasic (out‐of‐phase stimuli) DIN testing on a HearX Samsung Galaxy tablet with over‐the‐ear headphones, for a total of 128 measurements. DIN test results were compared with soundbooth audiometry using known adult "cut off criteria." Results: A logistic regression analysis adjusted for demographics (age, sex) and race was performed to compare CHL determination from DIN testing to CHL determination with soundbooth audiometry. The results showed 50% agreement with a p‐value of.753. The determinations based on combined DIN testing agreed with each other 33% of the time and had a p‐value of.373. Otologic pathology and age were not predictive of outcome. Conclusion: This preliminary analysis of DIN testing indicated that DIN and audiometric testing completed in a soundbooth were not significantly predictive of one another in the population of children aged 3–18 when using the adult cut‐off criteria for CHL differentiation. Given these findings, further testing is required in children to determine pediatric specific cut‐off values. [ABSTRACT FROM AUTHOR]

Introduction Cornelia de Lange Syndrome (CdLS) is a genetic disorder in which individuals may present sensorineural and/or conductive hearing loss, and the results of behavioral auditory assessments are not accurate. Objective To characterize the audiological profile of individuals with CdLS through behavioral, electroacoustic, and electrophysiological audiological assessments. Methods The study included 13 individuals of both sexes, aged between 3 and 26 years, with diagnoses confirmed through genetic studies. The following procedures were performed: medical history survey, otoscopy (pure-tone audiometry [PTA], speech audiometry, and acoustic immittance measures), and auditory brainstem response (ABR). Results In total 62.50% of the participants who underwent PTA had abnormal results (all of which were mild), with a predominance of bilateral conductive hearing loss (60%). Regarding tympanometry, 76.93% had abnormal results, most frequently type B (85.72% on the right and 88.89% on the left ear). Acoustic reflexes showed results compatible with tympanometry changes. Changes in ABR latency values compatible with middle-ear impairment were found in 8 of them (66.66%) – 3 had bilateral (37.50%), and 5 had unilateral impairments (62.50%). Conclusion Mild hearing loss was identified in 62.5% of the individuals with CdLS who underwent the behavioral audiological assessment. In the acoustic immittance measures, 76.9% of the participants presented a tympanometry curve characteristic of middle-ear changes. Acoustic reflexes were absent in 84.6% of the subjects. In the ABR, no changes were identified in auditory pathway integrity. On the other hand, changes in the absolute latency values were found, which are characteristic of conductive hearing loss. [ABSTRACT FROM AUTHOR]

Aim: This study is aimed to compare the outcomes of the two techniques (endoscopic single flap tympanoplasty- ESFT versus endoscopic double flap tympanoplasty - EDFT) of endoscopic tympanoplasty for repairing large tympanic membrane (TM)perforations with limited anterior remnant. Methodology: In this randomised controlled trial, forty patients with large sized TM perforations were included and randomised in the ESFT and EDFT arms with twenty patients in each group. Both the groups underwent endoscopic tympanoplasty using temporalis fascia graft. The graft uptake rates and hearing results were compared. Results: Graft success rate was 85% (17/20 cases) in the ESFT group and 90%(18/20 cases) in the EDFT group. The ABG (air-bone gap) improvement median with interquartile range(IQR) was 5 dB (3.12 dB-10 dB) in the ESFT group and 8.75 dB (5dB-11.87 dB) in the EDFT group. The difference was not statistically significant. Conclusions: In our study, there was no statistically significant difference in the graft success rate or hearing gain in ESFT or EDFT group. Moreover, there was longer operative time and need for an extra incision while raising the anterior flap. This lack of statistically significant results in our study may be since the study has a very small sample size. Whether the same conclusion is reproducible needs to be further explored by a larger sized randomised controlled trial. [ABSTRACT FROM AUTHOR]

Background: Otosclerosis is one of the most frequent causes of conductive hearing loss in adults. Surgical treatment is considered to be the gold standard for this disease. Endoscopic stapedectomy is a new approach for the treatment of otosclerosis that requires a bloodless field. Aim of the Study: To determine whether the routine use of β blockers as a premedication can improve the operative field in endoscopic stapedectomy or not. Patients and Methods: Sixty patients aged from 18 to 50 years, undergoing endoscopic stapedectomy, were included in this prospective, randomized, double blinded, placebo-controlled study. Patients were randomly assigned to receive either metoprolol 100 mg (group 1) or a placebo, vitamin tablet, (group 2) 60 min before surgery. Results: The age, gender, body weight and ASA physical status were comparable between the two groups. The average blood loss in the placebo group was significantly greater than in the metoprolol group, but the time of operation in the metoprolol group was not significantly shorter. The surgical field was assessed using the Fromme-Boezaart scale, and those who received metoprolol had considerably cleaner (p0.001) surgical fields. The mean arterial pressure (MAP) didn't differ statistically significant at basal measurement up to 30 min after anesthesia induction while it was significantly lower in the metoprolol group after 30 min of induction up to end of surgery (p < 0.001). The heart rate was also significantly lower (p < 0.001) in patients who received metoprolol from prior to anesthesia induction up to end of surgery. Conclusion: metoprolol significantly improves visual clarity and hemodynamics during endoscopic stapedectomy. [ABSTRACT FROM AUTHOR]

Bone Anchored Hearing Aids (BAHAs) are currently well-utilized for treatment of hearing-impaired patients with unilateral or bilateral mixed and conductive hearing loss as well as patients with single sided deafness. The objective of this study is to evaluate outcome of BAHA Attract system in terms of surgical, functional,audiological and cosmetic results This is a prospective observational study of 15 consecutive patients of single sided deafness (SSD) who qualified for and underwent BAHA Attract surgery at a tertiary care hospital over a duration of two years. Parameters analysed were: (1) Surgery and wound healing, (2) Post-operative functional results (Bern Benefit for Single sided deafness questionnaire (BBSSQ)), (3) audiological results (free field speech in noise audiometry in two situations: with signal from implanted side and from contralateral side), (4) Cosmetic results (introduction of novel Three Points Scoring System (TPSS). Clinico-radiological planning of surgical site for implant is also described.Mean surgical time was 39 minutes and healing was uneventful in 73 % of patients. All patients reported improvement in all ten hearing situations with maximum improvement for sound localization. Significant improvement of speech in noise was seen especially when signal was presented from implanted side. All patient had full score in TPSS for cosmesis except one patient. This was the same patient in whom clinico-radiologically planned site was discarded intraoperatively due to overpneumatised temporal bone.BAHA Attract system is safeand effective solution for single sided deafness with acceptable cosmesis. [ABSTRACT FROM AUTHOR]

Since 1952, myringoplasty has evolved with various surgical techniques, including overlay, underlay, sandwich, pegging, rosette, and plugging. Overlay and underlay methods are most commonly used. In 1998, Eavey introduced the cartilage and perichondrium butterfly inlay myringoplasty, designed for small to medium-sized perforation closure, offering practical advantages such as reduced surgical time and improved hearing outcomes. A one-year prospective study (September 2022 to September 2023) in a tertiary care hospital in India involved 30 patients aged 9–52 years with inactive small central perforations. Pre-operative pure tone audiometry (PTA) results were required to be no higher than 35 dB conductive hearing loss. General or local anesthesia was used based on the patient's age. The surgical technique involved visualizing the perforation, graft preparation, and transcanal insertion. Among the 30 patients, the graft uptake rate was 100% at 3 months and 93.33% at 6 months. Pre-operative mean PTA was 29.76 dB, which decreased to 25.03 dB post-operatively, with a statistically significant air-bone gap closure of 4.73 dB. Cartilage rosette inlay-onlay myringoplasty offers an effective solution for small to medium-sized perforations, achieving high success rates (93.33%). This technique is associated with reduced surgical time, suitability for day care surgery, and minimal scarring, making it a valuable addition to routine clinical practice. [ABSTRACT FROM AUTHOR]

The aim of the study was to evaluate and compare the efficacy of Endoscopic Adenoidectomy with Microdebrider over Conventional technique by Curettage using Impedance Audiometry findings. In this prospective, randomised, comparative study patients were divided into two groups to undergo one of the above adenoidectomy surgeries. Patients with symptoms of Adenoid hypertrophy after failed maximal medical therapy (12 weeks) were selected after due consideration of the inclusion and exclusion criteria. Baseline Impedance Audiometric assessment was done one week prior to surgery which was compared to Impedance Audiometric values 12 weeks post operatively in each group. This study enrolled 50 patients with adenoid hypertrophy, 25 patients of group A underwent Conventional adenoidectomy and the other 25 patients of group B underwent Endoscopic Adenoidectomy with Microdebrider. Significant difference in type of tympanogram before and after adenoidectomy was found in both the groups (p = 0.0008 in group A & p < 0.0001 in group B). In Group A Percentage of abnormal tympanograms becoming normal (Type A), 12 weeks after adenoidectomy was 80%. In Group B Percentage of abnormal tympanograms becoming normal (Type A), 12 weeks after adenoidectomy was 88%. A statistically significant improvement (p < 0.0001 in both the groups) in the percentage of ears with absent stapedial reflex was observed in both groups postoperatively, with no difference between the two groups suggestive of improvement in eustachian tube function. Association of Preoperatively & Postoperatively Eustachian tube function in each group undergoing adenoidectomy was statistically significant (p < 0.0001). Both forms of adenoidectomy are effective in managing adenoid hypertrophy with tubal dysfunction causing mild conductive hearing loss and prone for OME. However, the audiological and endoscopic evaluation seems to favor Endoscopic Adenoidectomy with microdebrider over conventional adenoidectomy, and it should be therefore considered in the therapeutic management of young patients with adenoidal disease. [ABSTRACT FROM AUTHOR]

Children in foster care have complexity around medical decision-making because of their unique custody status. When medical decision-making is necessary for a child in foster care, what perspectives are important? What if opinions are not aligned? Who makes the final decision for consent? This Ethics Rounds focuses on a young child in foster care who has had repeated ear infections. Foster caregivers and family of origin are not in agreement on the treatment plan. Two experts, a foster care pediatrician and a child welfare professional, comment on the important considerations for the case, including involvement of legal representation and best interest representation in medical decision-making as well as the variability in child welfare response by state. [ABSTRACT FROM AUTHOR]

Introduction: Bone anchored hearing implants (BAHI) are considered for conductive and mixed hearing loss, relying on osseointegration of a titanium implant. Limitations relate to constant skin contact, with resultant percutaneous infections and granulation. This study investigates whether patient characteristics and implant-specifications contribute to BAHIs' skin complications in a cohort with a uniform surgical approach. Methods: A 10 year (2014–2024) retrospective cohort study was conducted on BAHI procedures that were undertaken using a tissue-preserving 'punch' technique. Data on patient demographics, co-morbidities, implant type, surgical approach, and complications were collected. Poisson regression analysis was used to identify predictors of complications. Results: A total of 53 patients undergoing 55 BAHI surgeries by three ENT consultants were included. Factors that greatly increased implant-related percutaneous infections included the Cochlear™ BIA400 implant when compared to the Ponto™ BHX implant (twofold, CI 2.03–2.16), abutment sizes ≤ 10 mm (fourfold, CI 3.99–4.12) and male gender (9%, CI 1.07–1.12). Granulation episodes were affected by cardiovascular disease (CVD) status (1.5-fold, CI 0.26–0.78), BIA400 implant (threefold, CI 8.8.–9.2) and abutment sizes ≤ 10 mm (fourfold, CI 3.6–3.73). Revision surgery episodes increased with diabetic status (1.2-fold, CI 0.06–0.37) and abutment sizes ≤ 10 mm (threefold, 3.303–3.304). Conclusions: Larger cohort studies are required to confirm findings, particularly for implant and abutment size contributions. However, the findings suggest that using a larger abutment size when skin thickness meassuremets are borderline, improved hygiene education in male patients, pre-operative optimisation of CVD and diabetes, and adjusted patient follow-up based on risk stratification of the contributing factors to complication rates could reduce complication rates. [ABSTRACT FROM AUTHOR]

Background: The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4–9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures. Objective: To demonstrate our 40-plus years' experience in the management of PBC, depict the clinical features of PBC according to Sanna's Classification, evaluate the postoperative follow-up of surgically treated PBC patients, and determine the recurrence rate. Study design: Retrospective medical record review. Material and methods: Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated. Results: A total of 298 PBC cases were surgically treated at our center. The average age at presentation in this series was 47 years. Males are more affected than females with a male-to-female ratio of 2.2:1. The most common presenting symptoms were hearing loss (84%), tinnitus (48%), and facial nerve paralysis (45%). Mixed hearing loss (41%) was the commonest audiometric pattern of hearing loss followed by conductive hearing loss (26%) and profound sensorineural hearing loss (4%) and a total of 86 (29%) had anacusis at presentation. On preoperative facial nerve function examination, 133(45%) of patients had various degrees of paresis and complete paralysis whereas 55% had normal HB-I function. The commonest degree of paresis noted was HB-III (18%) followed by HB-VI (5%). A total of 150 (50%) patients had previous otologic surgery and two-thirds of these cases had two or more prior otologic surgeries. According to Sanna's PBC Classification system, we identified that the supralabyrinthine class (44%) is the commonest of all classes followed by massive (33%), infralabyrinthine-apical (9%), infralabyrinthine(8%), and apical (5%) classes in that order. However, only ten patients had congenital type of PBC. Extension to clivus, sphenoid, nasopharynx, intradural space, and occipital condyle was found in 8, 2, 1, and 2 cases respectively. The most commonly used surgical approaches at our center were TO, MTCA with rerouting of the facial nerve, and TLAB with external auditory canal (EAC) closure. Postoperative complications were minimal and the duration of follow-up ranged from one to 458 months with a mean duration of 65 months. Residual lesions were evident in 11 cases (3.7%), with the surgical cavity, middle and posterior fossa dura, and jugular bulb being the commonest sites. Conclusion: Petrous bone cholesteatoma represents diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. A high index of clinical suspicion, thorough clinical evaluation examination, and preoperative radiologic evaluation make the diagnosis easier. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is widely used to classify PBC in relation to the labyrinthine block. Radical disease removal should always come before hearing preservation. Cavity obliteration is the solution to the problems related to a large cavity. Finally, advancements in lateral skull base approaches create adequate surgical access for the complete removal of the lesion with excellent control of critical neurovascular structures. [ABSTRACT FROM AUTHOR]

Hearing loss has not been thoroughly investigated as a comorbidity in larger cohorts with neurofibromatosis type 1 (NF1). Available audiometric data were reviewed from patients with NF1 seen at a tertiary pediatric hospital to assess prevalence and risk factors for hearing loss. Of 1172 patients with NF1 seen between 2010 and 2022, 90 had available audiometric data and 48 of 90 patients (53%) had one or more audiogram revealing hearing loss. Those not referred to audiology were presumed to have normal hearing, resulting in a conservative hearing loss estimate of 4% for children and young adults with NF1. Of 90 patients with audiograms, 29 (32%) had conductive loss (CHL), 15 (17%) had sensorineural loss (SNHL), and 3 (3%) had mixed hearing loss. Hearing loss type was undetermined for one patient. For children with CHL, six had permanent CHL secondary to plexiform neurofibroma, 19 CHL were transient due to active middle ear dysfunction, and four CHL cases were indeterminate in etiology. For three children with SNHL or mixed hearing loss, etiology included history of ototoxic chemotherapy and/or family history of SNHL. In the 16 patients with SNHL or mixed hearing loss with more than one audiogram over time, progressive hearing decline was noted in eight of 16, and 26 of 178 hearing thresholds (15%) progressed. Our findings suggest that audiometric evaluations should be considered for at least a subset of children with NF1, given the higher-than-expected rate of hearing loss in patients with NF1 compared with the general population. [ABSTRACT FROM AUTHOR]

Background: Conductive or mixed hearing loss with an intact tympanic membrane is a group of diseases characterized by similar clinical symptoms. Definitive diagnosis depends on the findings of exploratory tympanic surgery. Cone-beam computed tomography (CBCT) has great potential for middle ear imaging. This study evaluated the diagnostic value of CBCT for conductive or mixed hearing loss with an intact tympanic membrane. Methods: CBCT and high-resolution computed tomography (HRCT) imaging data were collected from patients with an intact eardrum who received medical treatment in our hospital for conductive or mixed hearing loss from October 2020 to May 2023. The imaging characteristics and diagnostic values of CBCT and HRCT were analyzed. Results: A total of 137 patients who met the inclusion criteria and underwent CBCT were enrolled, including 89 with otosclerosis, 41 with ossicular chain interruption, and 7 with tympanosclerosis. CBCT clearly displayed a middle ear focus, such as low-density lesions located in the fissula ante fenestram, ossicular chain malformation or dislocation, and tympanic calcification foci. The area under the curve values for otosclerosis, ossicular chain interruption, and tympanic sclerosis were 0.934, 0.967, and 0.850, respectively. CBCT was more effective than HRCT for visualizing the lenticular process, incudostapedial joint, and stapes footplate. Conclusions: CBCT of the middle ear demonstrated higher-quality imaging to improve the diagnosis of conductive or mixed hearing loss with an intact tympanic membrane. Therefore, CBCT is recommended for further investigation of noninflammatory diseases of the middle ear with no special findings on HRCT. [ABSTRACT FROM AUTHOR]

Ossicular fixation disturbs the mobility of the ossicular chain and causes conductive hearing loss. To diagnose the lesion area, otologists typically assess ossicular mobility through intraoperative palpation. Quantification of ossicular mobility and evidence-based diagnostic criteria are necessary for accurate assessment of each pathology, because diagnosis via palpation can rely on the surgeons' experiences and skills. In this study, ossicular mobilities were simulated in 92 pathological cases of ossicular fixation as compliances using a finite-element (FE) model of the human middle ear. The validity of the ossicular mobilities obtained from the FE model was verified by comparison with measurements of ossicular mobilities in cadavers using our newly developed intraoperative ossicular mobility measurement system. The fixation-induced changes in hearing were validated by comparison with changes in the stapedial velocities obtained from the FE model with measurements reported in patients and in temporal bones. The 92 cases were classified into four groups by conducting a cluster analysis based on the simulated ossicular compliances. Most importantly, the cases of combined fixation of the malleus and/or the incus with otosclerosis were classified into two different surgical procedure groups by degree of fixation, i.e., malleo-stapedotomy and stapedotomy. These results suggest that pathological characteristics can be detected using quantitatively measured ossicular compliances followed by cluster analysis, and therefore, an effective diagnosis of ossicular fixation is achievable. [ABSTRACT FROM AUTHOR]

This study aimed to evaluate if ImageJ could be used to quantify the severity of external auditory exostoses (EAEs) in archaeological human skeletal remains. Thirty‐seven external auditory canals (EACs) with EAEs present (22 right, 15 left) of 100% completeness derived from individuals from archaeological sites on Cyprus dating from the Neolithic to the Roman period were available for analyses. EAC area and EAE area were measured three times as an intra‐observer error test using ImageJ in a two‐dimensional plane. The level of agreement between measurements was assessed using a paired T‐test, Bland–Altman plot, technical error of measurement, and coefficient of reliability. No statistically significant differences were found between measurements of EAC area and EAE area and a high technical measurement of error and coefficient of reliability resulted. ImageJ can be used with a high level of reliability for quantifying the severity of EAEs. This approach enables detailed quantification of EAEs in two‐dimensions, as well as specific measurement of EAE severity to evaluate differences between individuals and assemblages. The percentage of occlusion of the EAC by EAEs may contribute data to interpretations of the potential secondary clinical symptoms encountered by the individual, such as conductive hearing loss. This technique can only be applied to EACs that are of 100% preservation and in EACs where the EAEs are located laterally. Future studies should aim to incorporate the use of ImageJ for quantifying EAE severity in order to develop more detailed comparisons between individuals and assemblages in relation to their duration of exposure to wet and/or wet and windy environments and to evaluate the potential secondary symptoms that individuals with severe EAEs may have encountered. [ABSTRACT FROM AUTHOR]

Introduction: Bone conduction implants have been indicated for patients with conductive hearing loss, mixed hearing loss, and even profound unilateral sensorineural hearing loss. With the introduction of Bonebridge®, new transcutaneous implant options emerged. The latest is Osia®, a direct-drive variant or active systems, where the implant directly generates and applies vibration to the bone. Materials and Methods: Retrospective study of two cohorts of patients treated with active bone conduction implants at a single center, one with the Bonebridge® device and the other with Osia®. Outcomes: Fourteen patients were included, seven in each group (n = 14). The Bonebridge® group showed an average hearing gain in tonal intelligibility thresholds of 32.43 ± 21.39 dB and a gain in the average intelligibility threshold (with 50% discrimination) of 26.29 ± 19.10 dB. In the Osia® group, there was a gain in average tonal thresholds of 41.49 ± 14.16 dB and 23.72 ± 6.98 dB in average intelligibility thresholds. Both devices contributed to improvements in patients' quality of life, as assessed with APHAB in all the variables studied in the test. Both devices offer rehabilitation for hearing loss as an alternative to hearing aids. The Osia® system shows statistically significant (p < 0.05) improvements in mid and high frequencies, but Bonebridge® slightly outperforms in speech understanding at 50%. Differences in average tonal thresholds and quality of life are not statistically significant. Conclusions: While auditory improvement is observed postimplantation, other aspects, such as intelligibility thresholds and quality of life, lack statistical significance. Given the limited experience with Osia® and the small sample size, the choice of the device should be personalized. Although the literature is inconsistent due to small sample sizes and variable approaches, some studies suggest potential advantages of the Osia® system, especially in speech comprehension in different environments and greater hearing gain compared to Bonebridge®. [ABSTRACT FROM AUTHOR]

A speech intelligibility (SI) prediction model is proposed that includes an auditory preprocessing component based on the physiological anatomy and activity of the human ear, a hierarchical spiking neural network, and a decision back-end processing based on correlation analysis. The auditory preprocessing component effectively captures advanced physiological details of the auditory system, such as retrograde traveling waves, longitudinal coupling, and cochlear nonlinearity. The ability of the model to predict data from normal-hearing listeners under various additive noise conditions was considered. The predictions closely matched the experimental test data under all conditions. Furthermore, we developed a lumped mass model of a McGee stainless-steel piston with the middle-ear to study the recovery of individuals with otosclerosis. We show that the proposed SI model accurately simulates the effect of middle-ear intervention on SI. Consequently, the model establishes a model-based relationship between objective measures of human ear damage, like distortion product otoacoustic emissions, and speech perception. Moreover, the SI model can serve as a robust tool for optimizing parameters and for preoperative assessment of artificial stimuli, providing a valuable reference for clinical treatments of conductive hearing loss. [ABSTRACT FROM AUTHOR]

Introduction: Frequently, bone conduction hearing aids on a softband are not tolerated by children due to pressure on the head or visual esthetics. By way of contrast, a non-surgical hearing system - Adhear (Med-El, Innsbruck, Austria) - allows the sound processor to be attached using a noninvasive adhesive adapter. The objective of this study was to evaluate the effectiveness of the Adhear system and assess its subjective benefits in a child who had bilateral conductive hearing loss. Case report: The Adhear system was tested in a 13-year-old child with bilateral conductive hearing loss. Pure tone audiometry and speech audiometry in quiet were performed without and then with a pair of devices placed bilaterally. Word recognition scores (WRS) at 50 and 65 dB SPL in quiet were measured using the Pruszewicz monosyllabic Polish word test. After 4 weeks, subjective hearing benefit and experience with the Adhear system was done using the APHAB (Abbreviated Profile of Hearing Aid Benefit) questionnaire. Results: With Adhear, WRS at 50dB SPL increased significantly from an unaided score of 10% to 80%. The result of free-field audiometry with Adhear on both sides indicated a hearing level within the normal range. Conclusions: Adhear is an effective rehabilitation option for children with bilateral conductive hearing loss. [ABSTRACT FROM AUTHOR]

This article presents a case report on a patient who underwent a surgical procedure to repair a pseudomeningocele and tympanoplasty. The patient had symptoms of ear discharge and hearing loss. The surgical approach involved repairing a bony defect in the tegmen tympani using septal cartilage and fascia lata. The article emphasizes the importance of surgical treatment to prevent complications and discusses different surgical approaches for repairing tegmen defects. The combined middle cranial fossa approach is highlighted as a minimally invasive procedure that preserves hearing. [Extracted from the article]

Background: Recent technological evolutions allow for the identification of hearing level (HL) in infants soon after birth. The goal of early hearing detection and intervention is to maximize linguistic and communication skills and literacy development for children who are deaf or hard of hearing. Infants whose HL has been confirmed before 6 months of age must be provided early intervention. Aims and Objectives: The aim of the study was to observe the changes in the otoacoustic emissions (OAE) and brainstem auditory evoked potential (BAEP) in infants with birth asphyxia (BA). Materials and Methods: The present study was a case– control study. A total of 50 cases of infants with BA and 50 normal-term infants were part of the study after obtaining informed consent as per the ICMR guidelines. A through clinical examination and otoscopy examination were done to rule out any conductive hearing loss. Then, OAE and BAEP were recorded to estimate the hearing loss and hearing threshold of infants. Results: BAEP waveform I, III, and V absolute latencies were significantly prolonged among BA infants than normal infants. The interpeak latency (IPL) of the right ear III-V was significantly increased in BA infants. However, III-V and I-V latencies though prolonged in BA infants, they are not statistically significant. No significant interaural difference was noted in absolute and IPLs of both BA and normal infants. No significant relationship existed between the mode of delivery and the degree of hearing loss in both ears. Significant association was observed between the degree of hearing loss and severity of BA (hypoxic-ischemic encephalopathy) in both ears. Conclusion: BAEP recording in BA infants is useful to identify the hearing impairment at the earliest. The implication of this research work will help in early detection of hearing impairment, so that speech and language developmental delay in the child can be prevented. [ABSTRACT FROM AUTHOR]

Purpose: Bone conduction hearing devices (BCDs) that deliver sound across the skin (i.e., transcutaneous) are suitable for some individuals who have con- ductive or mixed hearing losses. Prescriptive targets for percutaneous devices are available, for example, from the Desired Sensation Level--Bone Conduction Hearing Device (DSL-BCD) algorithm. These targets, however, may require modification for use with transcutaneous BCDs. The current study investigated three key variables that may inform target modification: (a) comparison of thresholds measured using an audiometric bone conduction (BC) transducer versus transcutaneous BCDs that offer in situ threshold measurement, (b) trans- cutaneous BCD default force level outputs versus recommended DSL percuta- neous BC targets, and (c) the preferred listening levels (PLLs) of adults wearing transcutaneous BCDs in a laboratory setting. Method: Bilateral conductive hearing loss was simulated in 20 normal-hearing adults via earplugs. Thresholds were measured using a B-71 BC transducer and two commercially available BCDs coupled to a soft headband. DSL percu- taneous BC targets were generated, and PLLs were obtained for a 60-dB SPL speech stimulus. Force level outputs were measured using a skull simulator on the Audioscan Verifit2 at the hearing aids' default settings and at the partici- pants' PLL for each device. Results: On average, audiometric BC thresholds were significantly better than those measured in situ with each BCD. PLLs were similar to prescribed targets for one device with the smoother response shape and agreed in the high fre- quencies for both devices. Conclusions: In situ thresholds are significantly higher than audiometric BC thresholds, suggesting that device-based in situ measurement more accurately accounts for the signal transmission from transcutaneous BCDs. PLLs differed from the percutaneous targets and varied between devices, which may indicate that either target modifications or manipulations of device frequency response shaping are needed to approximate PLL with transcutaneous BCD devices. [ABSTRACT FROM AUTHOR]

Prevalence of hearing loss in children with autism spectrum disorder (ASD) is uncertain, as it is more challenging to assess hearing function in children with developmental difficulties (DD). We aimed to determine the prevalence and profiles of hearing loss in preschool children with ASD in a Southeast-Asian population who passed newborn hearing screening. A retrospective study of preschool children with DD (ASD, Global Developmental Delay (GDD), and Speech and Language Delay (SLD)) attending the Child Development Unit (CDU) at our hospital was performed. Three hundred and thirty-three children (ASD: n = 129; GDD: n = 110; and SLD: n = 94) underwent hearing assessments. Of these, 10.8% of children (n = 36, comprising 15 with ASD, 12 with GDD and 9 with SLD) had confirmed hearing loss. Hearing loss was predominantly bilateral in children with ASD and GDD; in those with SLD, unilateral and bilateral hearing loss were equally common. Conductive hearing loss occurred as frequently as sensorineural hearing loss in children with ASD and SLD, but was the dominant subtype in those with GDD. Moderate to severe hearing loss (n = 2) was noted only in children with ASD. Children with ASD and GDD required significantly more audiology visits and procedures to obtain conclusive hearing test results, compared to those with SLD. The need to identify hearing loss and monitor for resolution is particularly important in vulnerable populations with communication deficits, such as in those with ASD. [ABSTRACT FROM AUTHOR]

Hearing loss is the most prevalent neurosensory disorder in humans, with significant implications for language, social and cognitive development if not diagnosed and treated early. The present systematic review and meta-analysis aimed to determine the rate of hearing screening pass and genetic screening failure [universal newborn hearing screening (UNHS) pass/genetic failure] and to investigate the advantages of combining newborn hearing and genetic screening for newborn hearing impairment. The PubMed, Embase and Cochrane databases were searched from inception to September 2023 to identify studies reporting the combination of neonatal hearing screening with genetic screening. Duplicate literature, unpublished literature, studies with incomplete data, animal experiments, literature reviews and systematic studies were excluded. All the data were processed by STATA15.1 statistical software. A total of nine cross-sectional studies were included in this meta-analysis. The sample sizes ranged from 1,716 to 180,469, and there were a total of 377,688 participants. The pooled results revealed that the prevalence of passing the UNHS while failing genetic screening was 0.31% (95% CI, 0.22-0.41%). The prevalence of UNHS pass and gap junction protein beta 2 and solute carrier family 26 member 4 variant screen failure was 0.01% (95% CI, 0.00-0.02%) and 0.00% (95% CI, 0.00%), respectively, while the prevalence of mitochondrially encoded 12S RRNA variant screening failure and UNHS pass was 0.21% (95% CI, 0.18-0.26%). Combined screening has a significant advantage over pure hearing screening, especially in terms of identifying newborns with mitochondrial gene mutations that render them sensitive to certain medications. In clinical practice, decision-makers can consider practical circumstances and leverage the benefits of combined newborn hearing and genetic screening for early diagnosis, early counseling, and early intervention in patients with hearing loss. [ABSTRACT FROM AUTHOR]

Introduction: Bone conduction implants have been indicated for patients with conductive hearing loss, mixed hearing loss, and even profound unilateral sensorineural hearing loss. With the introduction of Bonebridge®, new transcutaneous implant options emerged. The latest is Osia®, a direct-drive variant or active systems, where the implant directly generates and applies vibration to the bone. Materials and Methods: Retrospective study of two cohorts of patients treated with active bone conduction implants at a single center, one with the Bonebridge® device and the other with Osia®. Outcomes: Fourteen patients were included, seven in each group (n = 14). The Bonebridge® group showed an average hearing gain in tonal intelligibility thresholds of 32.43 ± 21.39 dB and a gain in the average intelligibility threshold (with 50% discrimination) of 26.29 ± 19.10 dB. In the Osia® group, there was a gain in average tonal thresholds of 41.49 ± 14.16 dB and 23.72 ± 6.98 dB in average intelligibility thresholds. Both devices contributed to improvements in patients' quality of life, as assessed with APHAB in all the variables studied in the test. Both devices offer rehabilitation for hearing loss as an alternative to hearing aids. The Osia® system shows statistically significant (p < 0.05) improvements in mid and high frequencies, but Bonebridge® slightly outperforms in speech understanding at 50%. Differences in average tonal thresholds and quality of life are not statistically significant. Conclusions: While auditory improvement is observed postimplantation, other aspects, such as intelligibility thresholds and quality of life, lack statistical significance. Given the limited experience with Osia® and the small sample size, the choice of the device should be personalized. Although the literature is inconsistent due to small sample sizes and variable approaches, some studies suggest potential advantages of the Osia® system, especially in speech comprehension in different environments and greater hearing gain compared to Bonebridge®. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to review patient demographics, indications, intraoperative findings, complications/adverse events, and audiological outcomes related to the implantation of the Osia 2 device. Study Design: Retrospective case series. Setting: Single tertiary institutional experience. Methods: Patients who had undergone Osia 2 implantation by the senior author were identified from 2019 to present. Information was extracted from patient charts concerning patient demographics, indications for implantation, surgical findings, audiological outcomes, and adverse events. Results: Sixty patients and 67 implants were included. The median age was 51 years (R: 11‐92). Fifty‐five percent of patients had mixed hearing loss (HL), 30% had single‐sided deafness, and 15% had conductive HL. The mean operative time was 53.9 minutes. Only 4.5% of patients required bone polishing, and 3.0% required tissue thinning. The mean pure‐tone averages 4 gain from unaided conditions was 41.2 dB. Mean gain at 6 and 8 kHz from unaided conditions was 35.42 and 40.67 dB, respectively. Mean improvement in speech recognition threshold and word recognition score was significant in noise and quiet conditions. The all‐cause adverse event/complication rate in our series was 10.4%. The most common complications were infections (4.5%) and poorly controlled postoperative pain (3.0%). Hematomas occurred in 1.5% of patients. Reoperation was required in 4 patients; explant in 1. Conclusion: Use of the Osia 2 device in our series has resulted in good hearing outcomes, particularly in terms of high frequency gain. Complication rates were low. To our knowledge, this is the largest study to date reporting on Osia 2 outcomes. [ABSTRACT FROM AUTHOR]

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Unilateral Renal Agenesis (URA) is characterised by the absence of one kidney, and typically manifests asymptomatically due to compensatory hypertrophy of the remaining kidney. The condition is often detected incidentally because routine screening for renal anomalies is not universal globally. URA is generally associated with mixed or sensorineural hearing loss. A case involving contralateral conductive hearing loss is discussed in the present case report. The present case involves a eight-year-old female with isolated URA and no other concurrent abnormalities. The present report highlights an atypical audiological profile in URA, diverging (conductive type of hearing loss) from the commonly observed sensorineural or mixed hearing loss patterns. Understanding such variations contributes to the broader knowledge of the genetic and developmental links between the auditory and renal systems. This case underscores the importance of comprehensive audiological assessments in individuals with renal anomalies, potentially broadening diagnostic considerations and therapeutic approaches.

Bone conduction systems are sometimes not well tolerated due to the tension on the skull. Therefore, a brand non-surgical device was developed, the ADHEAR (MED-EL, Innsbruck, Austria). It is based on a novel fixation system using an adhesive adapter, which allows for a more comfortable and efficient mechanical transmission of the acoustic signal and that can be used as treatment for conductive hearing loss. The purpose of the study is to evaluate the performance of ADHEAR in two patients with unilateral conductive hearing loss. An 8-years-old patient with external ear canal stenosis obtained a functional gain of 28.75 dB, while a 16-years-old patient with Goldenhar syndrome showed a recovery of 53.75 dB. Therefore, both cases suggest that ADHEAR appears to be an effective treatment for pediatric patients who due to the medical condition cannot be treated surgically or fitted with conventional hearing aids.

Objective: To compare the rates of selected nervous system, cardiovascular, and otologic abnormalities in patients with and without Treacher Collins Syndrome (TCS). Design: Retrospective TriNetX platform cohort study. Setting: Aggregated and deidentified electronic health record (EHR) data from across the United States. Patients, Participants: Patients with TCS (n = 1,114) and a propensity matched control cohort without TCS (n = 1,114 matched from n = 110,368,585). Main Outcome Measured: Prevalence and relative risk (RR) of selected diagnoses in a propensity-matched cohort. Results: The RR of congenital malformations of the circulatory system in patients with TCS was 8.5 (95% CI 4.44-16.28). Patients with TCS also had higher rates of otologic abnormalities including conductive hearing loss (RR 44, 95% CI 24-83) and nervous system disorders including movement disorders (RR 2.60, 95% CI 1.27-5.50) and recurrent seizures (RR 4.2, 95% CI 2.12-8.33). Conclusions: We found a significantly elevated risk in TCS patients within all three systems. We postulate that the nervous system effects may be the result of one of the TCS-linked genes, for which a mutation has also been associated with progressive ataxia, cerebellar atrophy, hypomyelination, and seizures. As the previously-identified causal genes influence neural crest cells that form the head and face, these cells may also populate cardiac structures, resulting in cardiovascular abnormalities. Finally, the characteristic craniofacial abnormalities identified in TCS impair hearing and are associated with increased risk of otitis media. Our findings may help researchers to hypothesize the function of the genes underlying TCS, as well as to inform the care of affected individuals. [ABSTRACT FROM AUTHOR]

Abstract Background Hearing loss is a common problem worldwide with a global prevalence of 20%. There is no local relevant study from Iraq about the prevalence of hearing loss. Objective To assess the prevalence of hearing loss among patients attending the otolaryngology clinic, Ramadi city, Iraq. Method This retrospective study was conducted at the otolaryngology clinic, Ramadi city, Anbar government, Iraq. Patients with hearing loss were enrolled in the current study. Data were gathered from patients record regarding the age, sex, type, side, and severity of hearing loss. Additionally, the number of cases were registered for each month during the study period. Results Out of 8497, there were 2165 (25.48%) patients with hearing loss. The highest number of cases was registered in April (n = 246, 11.4%). The highest age group affected was 18–60 years (n = 1125) with slight male predominance (51.64%). The main cause of hearing loss was secretary otitis media (n = 762, 35.19/5). The vast majority of cases were with conductive hearing impairment (n = 2051, 94.73%). Unilateral hearing loss (50.76%) was slightly more than bilateral one. Mild hearing loss has outnumbered (n = 1905, 88%) the other severities. Conclusion The prevalence of hearing loss was 25.48%. Hearing loss could affect any age with slight male predominance. The majority of the cases were with conductive type and mild severity.

Background: Mutations in Wolfram syndrome 1 (WFS1) cause Wolfram syndrome and autosomal dominant non-syndromic hearing loss DFNA6/14/38. To date, more than 300 pathogenic variants of WFS1 have been identified. Generally, the audiological phenotype of Wolfram syndrome or DFNA6/14/38 is characterized by low-frequency hearing loss; however, this phenotype is largely variable. Hence, there is a need to better understand the diversity in audiological and vestibular profiles associated with WFS1 variants, as this can have significant implications for diagnosis and management. This study aims to investigate the clinical characteristics, audiological phenotypes, and vestibular function in patients with DFNA6/14/38. Methods: Whole-exome or targeted deafness gene panel sequencing was performed to confirm the pathogenic variants in patients with genetic hearing loss. Results: We identified nine independent families with affected individuals who carried a heterozygous pathogenic variant of WFS1. The onset of hearing loss varied from the first to the fifth decade. On a pure-tone audiogram, hearing loss was symmetrical, and the severity ranged from mild to severe. Notably, either both low-frequency and high-frequency or all-frequency-specific hearing loss was observed. However, hearing loss was non-progressive in all types. In addition, vestibular impairment was identified in patients with DFNA6/14/38, indicating that impaired WFS1 may also affect the vestibular organs. Conclusions: Diverse audiological and vestibular profiles were observed in patients with pathogenic variants of WFS1. These findings highlight the importance of comprehensive audiological and vestibular assessments in patients with WFS1 mutations for accurate diagnosis and management. [ABSTRACT FROM AUTHOR]

Background: The adenoids act as a reservoir of bacterial pathogens and immune molecules, and they are significantly involved in children with otitis media with effusion (OME). As an essential carrier of intercellular substance transfer and signal transduction, exosomes with different biological functions can be secreted by various types of cells. There remains significant uncertainty regarding the clinical relevance of exosomes to OME, especially in its pathophysiologic development. In this study, we will seek to determine the biological functions of exosomes in children with adenoid hypertrophy accompanied by OME (AHOME). Methods: The diagnostic criteria for OME in children aged 4–10 years include a disease duration of at least 3 months, type B or C acoustic immittance, and varying degrees of conductive hearing loss. Adenoidal hypertrophy is diagnosed when nasal endoscopy shows at least 60% adenoidal occlusion in the nostrils or when nasopharyngeal lateral X-ray shows A/N > 0.6. Children who meet the indications for adenoidectomy surgery undergo adenoidectomy. Peripheral blood, nasopharyngeal swab, and adenoid tissue will be collected from patients, and the exosomes will be isolated from the samples. Following the initial collection, patients will undergo adenoidectomy and peripheral blood and nasopharyngeal swabs will be collected again after 3 months. Expected results: This study aims to identify differences in exosomes from preoperative adenoid tissue and peripheral blood samples between children with AHOME and those with adenoid hypertrophy alone. Additionally, it seeks to determine changes in microbial diversity in adenoid tissue between these groups. Conclusions: The findings are expected to provide new insights into the diagnosis and treatment of OME, to identify novel biomarkers, and to enhance our understanding of the pathophysiology of OME, potentially leading to the development of innovative diagnostic and therapeutic approaches. [ABSTRACT FROM AUTHOR]

This study investigated sound localization abilities in patients with bilateral conductive and/or mixed hearing loss (BCHL) when listening with either one or two middle ear implants (MEIs). Sound localization was measured by asking patients to point as quickly and accurately as possible with a head-mounted LED in the perceived sound direction. Loudspeakers, positioned around the listener within a range of +73°/−73° in the horizontal plane, were not visible to the patients. Broadband (500 Hz–20 kHz) noise bursts (150 ms), roved over a 20-dB range in 10 dB steps was presented. MEIs stimulate the ipsilateral cochlea only and therefore the localization response was not affected by crosstalk. Sound localization was better with bilateral MEIs compared with the unilateral left and unilateral right conditions. Good sound localization performance was found in the bilaterally aided hearing condition in four patients. In two patients, localization abilities equaled normal hearing performance. Interestingly, in the unaided condition, when both devices were turned off, subjects could still localize the stimuli presented at the highest sound level. Comparison with data of patients implanted bilaterally with bone-conduction devices, demonstrated that localization abilities with MEIs were superior. The measurements demonstrate that patients with BCHL, using remnant binaural cues in the unaided condition, are able to process binaural cues when listening with bilateral MEIs. We conclude that implantation with two MEIs, each stimulating only the ipsilateral cochlea, without crosstalk to the contralateral cochlea, can result in good sound localization abilities, and that this topic needs further investigation. [ABSTRACT FROM AUTHOR]

Background/Objectives: Otosclerosis is a relatively uncommon condition that causes conductive hearing loss in children. The preferred treatment for adults is stapedotomy, while for individuals under 18 years old, there is an ongoing discussion about the best treatment approach. Thus, the surgical procedure for the stapes in pediatric patients continues to be a subject of debate. This study aimed to evaluate the results of stapes surgery in children, trying to understand, based on our results, whether this is actually the most suitable option. Methods: The study included 18 patients who underwent surgery between January 2013 and December 2023. The patients' ages ranged from 11 to 18 years, with an average age of 14.7. Out of the total 21 surgeries, three patients opted for bilateral surgery. Pre- and post-operative data were compared, focusing on the mean air conduction (AC) and bone conduction (BC) thresholds at frequencies of 0.5, 1, 2, and 4 kHz. Additionally, pre-operative thresholds and the post-operative air–bone gap (ABG) were examined. Results: After a year, the air–bone gap was effectively reduced to 10 dB or less in 94% of the 21 cases, and to 20 dB or less in 98% of all cases. Conclusions: Our results and research in the field have consistently shown that stapedotomy, when conducted by skilled otosurgeons, is a reliable and successful procedure for a considerable number of patients. The outcomes it generates are similar to those achieved through the procedure conducted during adulthood. [ABSTRACT FROM AUTHOR]

Background: Enlarged vestibular aqueduct (EVA) syndrome can mimic otosclerosis in adults, presenting with an air–bone gap (ABG) and even absent stapedial reflexes. The ABG in inner-ear disorders is currently the object of several authors' studies and seems to be related to a third mobile window (TMW) phenomenon. This can lead to misdiagnosis and inappropriate treatment. Given that it would be inappropriate and harmful to perform CT scans in all patients with a clinical diagnosis of otosclerosis, this study aims to highlight some clinical features useful for the differential diagnosis between otosclerosis and these rare cases of EVA presenting with an ABG, thus enabling the identification of suspected cases to be tested with CT scans. Methods: Between April and May 2024, a narrative review was conducted focusing on the differential diagnosis between some rare cases of EVA and otosclerosis. Clinical, audiological, and radiologic features of both conditions were investigated. Results: This review demonstrates the diagnostic challenge in differentiating atypical cases of EVA from otosclerosis in a subset of patients. Clinical and audiological features are important for differential diagnosis, but may not always be sufficient. Therefore, high-resolution computed tomography (HRCT) of the temporal bone plays a pivotal role in definitive diagnosis. Conclusions: In some specific cases, pre-operative imaging assessment using HRCT emerges as an essential tool for differentiating these two conditions and avoiding unnecessary stapes surgery. [ABSTRACT FROM AUTHOR]

Programmable variable pressure valves were introduced in the 1980s, providing a non-invasive solution to post-operative alterations of the valve opening pressure to address problems of under or overdrainage. Since their increased use in the treatment of hydrocephalus, there have been case reports of unintentional alterations of the valve opening pressure following exposure to magnetic fields in everyday environments, from televisions to rollercoasters. Here we describe two cases of patients' programmable valves being altered following audiology assessments. We subsequently discuss some of the available research on the safety of these valves in various magnetic field strengths, alongside interim and updated recommendations made by the British Society of Audiologists with regards to testing of children with programmable shunts in place. [ABSTRACT FROM AUTHOR]

The infrafloccular approach in microvascular decompression (MVD) surgery for hemifacial spasm (HFS) was investigated for the prevention of hearing complications. Retrospective analysis of 136 patients who underwent MVD for HFS in 2019. The infrafloccular approach for MVD was adopted to resolve the symptom and protect hearing function. Postoperative hearing function was evaluated subjectively and objectively within 30 postoperative days, and later mainly subjectively. Postoperative condition of mastoid air cells based on the CT scan of the following day was also recorded. No final postoperative subjective hearing impairment was found in all patients. Mastoid air cells were opened in 105 patients, and subjective hearing impairment was recorded in 57 patients within 30 postoperative days, but all improved later. Increases in thresholds of greater than 10 dB in air conduction were observed in 28 patients, including 26 of transient conductive hearing loss (increases in the threshold of less than or equal to 10 dB). The other 2 patients with increases in the threshold of greater than 10 dB in both air and bone conduction had improvement confirmed by later audiometry. Infrafloccular approach in MVD for HFS provides a good hearing outcome. Inclusion of postoperative hearing conditions in the later period is ideal for a precise hearing evaluation. [ABSTRACT FROM AUTHOR]

Background: Bone-anchored hearing instruments (BAHIs) are an important alternative solution for hearing loss in a particular group of patients. This study aims to examine shifts in attitudes about BAHIs among hearing health professionals over time. Methods: A survey was designed to assess the knowledge and attitude of otorhinol aryngologists (ENT) and audiologists (Au) concerning BAHIs. The survey was administered in 2019 and 2023 at various conferences. Results: The survey received 243 responses (122 in 2019 and 121 in 2023). Among these, 186 (77%) were from ENTs, while 49 (20%) were from Aus. Notably, the preference for using BAHI for conductive and mixed hearing loss cases increased from 19.3% to 47.9%. The number of individuals with prior experience decreased, yet those who applied BAHIs grew. Signifcant shifts were observed in patient selection based on hearing level, hearing loss type, and clinical condition. The profession and experience emerged as infuential factors in these changes. Conclusion: While most subjects recognized the signifcance of hearing rehabilitation, their fundamental knowledge and familiarity with BAHIs remained limited. A broader implementation of educational and training initiatives focusing on BAHIs is essential to enhance understanding and profciency in this area. [ABSTRACT FROM AUTHOR]

Aim: 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. Materials and methods: 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. Results: 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). Conclusion: 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. [ABSTRACT FROM AUTHOR]