[Otosclerosis : clinical presentation and management]. / Otosclérose : présentation clinique et prise en charge.

Otosclerosis is characterized by pathological remodeling of the bone of the otic capsule. Classically, foci of pathological bone leads to progressive fixation of the stapes, resulting in conductive hearing loss. Involvement can be uni- or bilateral. Otoscopy is normal. -Hypoacusis generally appears between the age of 30 to 50, regularly with tinnitus. Sometimes, the inner ear is also affected, with sensori-neural hearing loss or vestibular impairment (vertigo). Successful hearing rehabilitation can be achieved with hearing aids or stapes surgery (stapedotomy). Cochlear implantation is considered in cases of severe or profound sensorineural hearing loss. High resolution CT-scan or Cone Beam CT confirm the diagnosis and optimal planning of surgical treatment.

L'otosclérose est caractérisée par un remodelage pathologique de l'os de la capsule otique. Classiquement, un foyer d'os patho­logique entraîne une fixation progressive de l'étrier qui résulte en une atteinte auditive de transmission. L'atteinte peut être uni ou bilatérale. L'otoscopie est normale. L'hypoacousie apparaît généralement entre 30 et 50 ans, régulièrement avec un acouphène. Parfois, l'oreille interne est aussi affectée, avec une atteinte auditive de perception ou encore vestibulaire (vertige). La réhabilitation auditive apporte d'excellents résultats, par un appareillage auditif ou une chirurgie de l'étrier, la stapédotomie. En cas d'atteinte auditive de perception marquée, une implantation cochléaire est considérée. Un CT-scan ou un Cone Beam CT en coupes fines permet de confirmer le diagnostic et de planifier la chirurgie.

Refractory Chronic Diffuse Granular Myringitis with Medial Meatal Stenosis.

Granular myringitis is a chronic inflammatory condition of the tympanic membrane that does not involve the middle ear. Various treatment modalities have been proposed for the treatment of granular myringitis, but there is no standard treatment regime. A 60-year-old woman had left persistent ear discharge for 4 months. Examination revealed diffuse granulation tissue, forming a pseudomembrane at the medial aspect of the ear canal and obstructing the tympanic membrane. An audiogram revealed mild-to-moderate left-sided conductive hearing loss. She was treated with multiple courses of ear drop antibiotics but had no improvement. The decision for surgical intervention was driven by the presence of a grade IV medial meatal stenosis, the potential risks associated with prolonged medical management, the distressing impact on the patient's life, and a shared decision-making process. A combined transcanal and postauricular endoscopic approach whereby excision of the granulation tissue, canalplasty, and myringoplasty were performed. She exhibited complete symptom resolution and reported an improved quality of life. This approach yielded successful symptom resolution, highlighting its potential in managing refractory chronic granular myringitis. We aimed to carefully weigh the risks of surgery against its potential benefits in a refractory chronic case, acknowledging the inherent risks and disadvantages of surgical interventions. Further studies are warranted to evaluate the long-term outcomes and benefits of this approach.

Traumatic Tympanic Membrane Perforation in Children: A Descriptive Cross-sectional Study.

INTRODUCTION:   TTraumatic perforation of the tympanic membrane in pediatric population is often encountered in primary  otolaryngologic clinics or in the emergency department. The objective of the  study was to find out the clinical presentation of traumatic tympanic membrane perforation in the pediatric population. METHODS: A cross-sectional study was done from February, 2023 to February, 2024 after obtaining the ethical approval from the Institutional Review Board (2023/114). All the patients aged less than 18 years and presenting with traumatic tympanic membrane perforation were included in the study. Collected data was entered and analysed using Microsoft Excel. Patients were evaluated for demographics, mechanism of trauma and clinical characteristics of ear drum perforation in children. RESULTS: A total of 384 children aged less than 18 years were included in the study among which 267 (69.53%) were males. Physical assault 109 (28.39%) was the most common cause of tympanic membrane perforation. The most common symptom at the time of presentation was ear block/hearing loss 208 (54.16%). Conductive hearing loss was present in 214 (55.73%) children. CONCLUSION: Traumatic perforation of the tympanic membrane resulted mostly from the blunt force trauma especially in male children.

Expert opinion on candidacy for bone conduction hearing implants Osia System and Baha Connect System.

<b>Introduction:</b> Bone conduction hearing implants (BCHI) are a widely used rehabilitation solution for patients with conductive hearing loss (CHL), mixed hearing loss (MHL), or single-sided deafness (SSD).<b>Aim:</b> This expert review presents candidacy criteria considerations when choosing between active transcutaneous bone-conduction hearing devices (Osia System) and passive percutaneous bone-conduction hearing devices (Baha Connect System) to help streamline the decision-making process in those contexts where economics have a major impact on professionals' and patients' choice.<b>Methods:</b> Eight experts participated in two online surveys and two virtual meetings to discuss real-world clinical experience to highlight treatment approaches and factors considered when counseling the patients and selecting an optimal BCHI solution. Key considerations for decision-making were recorded following consensus from all experts.<b>Conclusions:</b> Aspects in decision making include the requirement to use local <i>versus</i> general anesthesia for the implantation procedure, bone thickness, considerations for future magnetic resonance imaging (MRI) procedures, and patient preference. Increased risk of skin infections, requirements for cleaning and managing the implant site, particularly for those with limited dexterity, as well as esthetic concerns could make the Baha Connect System unsuitable for some patients. In these cases, the Osia System may provide clear advantages, particularly in patients for whom good hearing performance is a priority, and this would need to be discussed individually with the patient in a multidisciplinary setting. Conversely, for patients requiring minimally invasive surgery, who have contraindications for general anesthesia or require frequent head MRI scans in the future, the Baha Connect System may be more suitable.

The impact of the Cochlear™ Osia® 2 System on patients with mixed or conductive hearing loss: A comparison with Cochlear™ Baha® Attract System outcomes.

OBJECTIVE: This prospective study assessed the efficacy of the Cochlear™ Osia® 2 System compared to the previous Baha® Attract System in patients with mixed or conductive hearing loss (MHL/CHL). METHODS: In this prospective case-control study, 10 patients (2 men and 8 women) with MHL/CHL were implanted with the Osia® 2 System. Their audiological outcomes were compared with 13 patients (2 men and 11 women) who had previously been implanted with the transcutaneous Baha® Attract system. We compared the complications and compliance of the two groups. Also, in the Osia 2 System group, subjective satisfaction was assessed using the Korean version of the International Outcome Inventory for Hearing Aids (K-IOI-HA) questionnaire and the Abbreviated Profile of Hearing Aid Benefit (APHAB) questionnaire. RESULTS: Complications such as poor magnetization, pain & infection, and abnormal noise were more common in the Baha Attract group, although not statistically significant. Also, the Osia 2 group exhibited better compliance. Subjective satisfaction was assessed using the K-IOI-HA and APHAB questionnaires with the Osia 2 group, revealing significantly improved scores in ease of communication, reverberation, background noise, and higher K-IOI-HA scores post-implantation. Postoperative-aided thresholds with both systems were significantly lower than preoperative-unaided thresholds, with the Osia 2 System demonstrating notably high satisfaction levels. Although both systems showed similar preoperative and postoperative word-recognition scores, the Osia 2 System provided greater audiological gain, especially at 2 kHz and 4 kHz frequencies. Additionally, the functional gain of both systems was comparable across all frequencies. CONCLUSIONS: The Osia 2 System demonstrated high subjective satisfaction and improved audiological outcomes compared to the Baha Attract system in patients with conductive or mixed hearing loss. Its superior audiological gain, particularly at critical frequencies, along with better compliance, suggests it as a favorable option for this patient population.

Classification of ossicular fixation based on a computational simulation of ossicular mobility.

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.

[Treacher Collins Syndrome 2 caused by a novel pathogenic variant in PLOR1D: clinical report and literature review].

Objective: To investigate the clinical features, molecular etiology, and treatment of a family with Treacher Collins Syndrome 2 (TCS2). Methods: Information of the proband (female, 8 years old) including medical history and family history was collected. Physical examination and examinations concerning laboratory, audiology, and radiology were performed on the proband. Physical examination was also performed on the family members. Genomic DNA of proband was extracted for whole exome sequencing, and then the genomic DNA of family members was extracted for Sanger sequencing. POLR1D and TCS2 related literatures published before August 31,2023 were searched and sifted in PubMed and CKNI databases. The clinical characteristics of TCS2 were summarized. Results: The proband had poor hearing since childhood, with pure tone audiometry indicating conductive hearing loss. She had a smaller jaw, bilateral preauricular fistulas and cup-shaped ear deformities. Temporal bone CT scan revealed deformities in the left external ear canal, bilateral middle ear and inner ear. A bone-conduction hearing aid device was surgically implanted, resulting in restoration of almost normal hearing levels. The proband's mother also had a slightly smaller jaw. Genetic analysis revealed a novel heterozygous variant NM_015972.4:c.38_47del in the POLR1D gene in the proband, which was inherited from her mother. A review of the literature revealed no clear evidence of genotype-phenotype correlation in TCS2. Conclusions: Molecular diagnosis plays a vital role in the diagnosis of TCS2. Patients with normal facial phenotype may be carriers of pathogenic variants in the POLR1D gene and have the risk of passing it to the offsprings with complete penetrance. Proper bone conductive hearing devices can improve the quality of life of TCS2 patients.

A Systematic Review of Surgical Characteristics and Adverse Events of an Active, Transcutaneous Bone Conduction Device.

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.

Diagnostic Value of Cone-Beam Computed Tomography in Conductive or Mixed Hearing Loss with Intact Tympanic Membrane.

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.

Beyond the surface: exploring contributing factors to bone anchored hearing implant complications.

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.

P1-N1-P2 Cortical Auditory Evoked Potentials in Chronic Unilateral Acquired Conductive Hearing Loss in Adults.

BACKGROUND:  Chronic unilateral hearing loss causes imbalanced auditory input to the brain that triggers cortical reorganization. The effect of sensorineural hearing loss on the central auditory system (CAS) has been thoroughly studied, while there is a paucity of research on the effect of conductive hearing loss (CHL). The aim of this study was to assess the P1-N1-P2 cortical auditory evoked response potential (CAEP) in adult individuals with chronic acquired unilateral CHL. METHODS:  This study included 108 participants of both genders: 54 patients with unilateral chronic CHL who were compared to well-matched 54 controls. All were subjected to history-taking, otologic examination, basic audiological evaluation, and bone conduction N1-P2 CAEP. RESULTS:  The affected ears of the cases showed highly statistically significant shorter CAEPs N1, P2, N1-P2 latencies but not P1, and showed highly statistically significant larger N1, P2, N1P2, amplitude than the control group. Latencies decreased and amplitudes increased as the degree of CHL increased, but were not affected by patients' age, side, or duration of the CHL. Cases with tinnitus had statistically significant and worse results than those without tinnitus. CONCLUSION:  Unilateral chronic CHL might enhance neurocortical plasticity, with greater changes occurring at greater degrees of the CHL.

Conductive Hearing Loss Associates With Dementia, and Middle Ear Reconstruction Mitigates This Association: A Multinational Database Study.

OBJECTIVE: To test the hypothesis that conductive hearing loss (CHL) is associated with dementia, and that middle ear reconstruction (MER) associates with improved outcomes for these measures in a multinational electronic health records database. STUDY DESIGN: Retrospective cohort study with propensity-score matching (PSM). SETTING: TriNetX is a research database representing about 110 million patients from the United States, Taiwan, Brazil, and India. PATIENTS: Subjects older than 50 years with no HL and any CHL (ICD-10: H90.0-2). Subjects of any age with and without any MER (CPT: 1010174). MAIN OUTCOME MEASURES: Odds ratios (ORs) and hazard ratios with 95% confidence intervals (95% CIs) for incident dementia (ICD-10: F01, F03, G30). RESULTS: Of 103,609 patients older than 50 years experiencing any CHL, 2.74% developed dementia compared with 1.22% of 38,216,019 patients with no HL (OR, 95% CI: 2.29, 2.20-2.37). Of patients experiencing CHL, there were 39,850 who received MER. The average age was 31.3 years, with 51% female patients. A total of 343,876 control patients with CHL were identified; 39,900 patients remained in each cohort after 1:1 PSM for HL- and dementia-related risk factors. Matched risk for developing dementia among MER recipients was 0.33% compared with 0.58% in controls (OR: 0.58, 0.46-0.72). CONCLUSIONS: CHL increases the odds for dementia, and MER improves the odds for incident dementia. This study represents the first population study on the topic of CHL, MER, and dementia.

Evaluation of Clinical Performance of Ponto Implantation Using a Minimally Invasive Surgical Technique-A Prospective Multicenter Study.

OBJECTIVE: To investigate the clinical outcomes of bone-anchored hearing implant surgery using the MONO procedure. STUDY DESIGN: Multicenter, multinational, single-arm, prospective trial with a 12-month follow-up. SETTING: Seven European university hospitals from the United Kingdom, Sweden, Denmark, and The Netherlands. PATIENTS: Fifty-one adult patients requiring surgical intervention for bone conduction hearing. INTERVENTION: Bone-anchored hearing implant surgery using the MONO procedure. MAIN OUTCOME MEASURES: The primary endpoint assessed implant usability 3 months after surgery. Implant status, soft tissue reactions, pain and numbness, postoperative events, and sound processor usage were assessed at all follow-up visits. Hearing-related quality of life was evaluated using the Glasgow Benefit Inventory (GBI). RESULTS: At 3 months, 94.2% of the implant/abutment complexes provided reliable anchorage for sound processor usage. No severe intraoperative complications occurred. Sixty-nine percent of surgeries were performed under local anesthesia, with surgery lasting 10 minutes on average. Four implants were lost due to trauma (n = 2), spontaneous loss of osseointegration (n = 1), or incomplete insertion (n = 1). Adverse soft tissue reactions occurred in 2.6% of visits, with a maximum Holgers grade of 3 (n = 1) and grade 2 (n = 5) across patients. Hearing-related quality of life at 3 months improved in 96% of patients. CONCLUSION: The MONO procedure provides a safe and efficient surgical technique for inserting bone-anchored hearing implants with few and minor intra- and postoperative complications.

Feasibility and Outcomes of an Active Osseointegrated Bone Conduction Implant in Children as Young as 5 Years of Age.

INTRODUCTION: This study reviews the feasibility of implanting active osseointegrated bone conduction devices in young children, below the prior age for FDA indication (<12 years), which has recently been reduced to 5 years. Outcomes included differences in adverse event rates and operative time between two groups (<12 and 12 years or older). MATERIALS AND METHODS: This study is a retrospective review of children receiving active osseointegrated bone conduction devices at a tertiary referral center academic hospital. One hundred and twenty-four children received 135 active osseointegrated bone conduction devices (May 2018-March 2024). RESULTS: Of 135 devices, 77 (57%) were in children <12 years (mean age (SD) = 7.9 (2.0) years, range = 4.9-11.9 years) and 58 (43%) were in 12 years or older (mean age (SD) = 15.1 (1.7) years, range = 12-18 years). Adverse events were significantly higher in the older group, occurring in 8 (10%) of 77 devices in children <12 years and 15 (26%) of 58 devices in children 12 years and older (26%) (Fisher's exact test = 0.0217 at p < 0.05). Major adverse events occurred in 5/124 (4%) patients, with 2 in patients <12 years (2/73, 3%) and 3 in children 12 and older (3/51, 6%). The proportion of major events between groups was not significantly different (Fisher's exact test = 0.4, p < 0.05). Mean surgical time was significantly less (t = -2.8799, df = 120.26, p = 0.005) in the children <12 years (mean (SD) = 66.5 (22.4) min) compared to those 12 and over (mean (SD) = 78.32 (23.1) min). CONCLUSIONS: Implantation of active osseointegrated bone conduction devices is feasible in children as young as 5 years and demonstrates low rates of complication. Further miniaturization may allow even earlier safe intervention.

The Merits of Bilateral Application of Middle Ear Implants in Patients With Bilateral Conductive and/or Mixed Hearing Loss.

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.

[Analysis of the efficacy of fiber laser stapes fenestration in the treatment of 58 cases of otosclerosis and ossicular malformation].

Objective: To explore the surgical efficacy of conductive deafness caused by otosclerosis and ossicular malformation with 980 nm fiber laser stapedial floor fenestration. Methods: Data of 58 patients (ears) who were diagnosed with conductive deafness caused by otosclerosis (49 ears) and ossicular malformation (9 ears) treated by 980 nm Diode laser small-fenestra stapedotomy were retrospectively analyzed. Air conduction (AC) thresholds, bone conduction (BC) thresholds, and air-bone gap (ABG) at 0.5, 1, 2, 4 kHz pure tone frequencies were compared before and 3 months after surgery, and the advantages and precautions of 980 nm fiber laser were summarized. Paired t-test (SPSS 26.0 software) was use to analyze the listening data. Results: Fiber optic laser stapes fenestration and artificial stapes implantation were successfully completed in all 57 cases (ears), the hearing of another one patient (ear) with floating malformation of detachment of stapedial floor was lower than that before surgery. Preoperative at 0.5, 1, 2, 4 kHz frequencies of AC thresholds, BC thresholds, and ABG were (65.4±9.7) dB, (27.2±8.9) dB, and (38.2±9.8) dB respectively. Postoperative 3 months at the same frequency of AC thresholds, BC thresholds, and ABG were (42.1±11.3) dB, (26.9±6.6) dB, and (15.2±9.1) dB. Preoperative and postoperative of AC threshold and ABG were statistically significant at 0.5, 1, 2, 4 kHz (t value was 13.270 and13.948, both P<0.01), and yet the BC threshold was not statistically significant before and after surgery at the same frequency (t=0.418, P>0.05). Conclusions: 980 nm fiber laser stapes floor fenestration is an effective treatment for conductive deafness caused by otosclerosis and ossicular malformation.

Distinct audiometric patterns in congenital round window atresia: A comparative study with common congenital middle ear anomalies.

OBJECTIVE: Little is known about the hearing characteristics in patients with congenital round window atresia (CRWA). This study aimed to investigate hearing characteristics in patients with CRWA by comparing them with two relatively common congenital middle ear anomalies: congenital stapedial fixation (CSF) and congenital ossicular discontinuity (COD). METHODS: Literature searches yielded five patients with surgically confirmed CRWA (seven ears), who were included in the CRWA group, along with one of our patients. Air and bone conduction thresholds; air-bone gap (ABG); and presence and depth of the Carhart notch were analyzed. These audiometric variables in the CRWA group were compared with those in the CSF (n = 15) and COD (n = 22) groups, comprising patients identified from our institution's medical database. RESULTS: Average bone and air conduction thresholds in the CRWA group were 16.4 (standard deviation [SD]: 2.9; 95 % confidence interval [CI]: 14.6-18.3) and 44.6 (SD: 3.5; 95 % CI: 42.6-47.3) dB hearing level (HL). Bone conduction thresholds at high frequencies (≥2 kHz) were higher than those at low frequencies (<2 kHz), while air conduction thresholds at high frequencies were lower than those at low frequencies: ABGs at high frequencies were significantly smaller than those at low frequencies (2 kHz vs. 0.5 kHz, p = 0.027; 2 kHz vs. 1 kHz, p = 0.041; 4 kHz vs. 0.5 kHz, p = 0.042; 4 kHz vs. 1 kHz, p = 0.027). There were no between-group differences in incidence and depth of the Carhart notch. CONCLUSION: CRWA could manifest as a distinct audiometric pattern with poorer bone conduction and better air conduction at ≥2 kHz, resulting in significantly smaller ABGs at higher frequencies than that at lower frequencies. Our findings indicated that this pattern differed from that of CSF and COD. The unique beer bottle-shaped audiogram associated with CRWA might facilitate its early diagnosis in patients with congenital conductive hearing loss.

[An unusual case: fracture of the handle of the malleus due to finger manipulation]. / Ein ungewöhnlicher Fall: Fraktur des Hammergriffs durch Fingermanipulation.

An isolated fracture of the handle of the malleus is a rare entity in otorhinolaryngology and manifests clinically as acute-onset unilateral hearing loss. Several factors may cause this injury, including acute barotraumatic pressure changes or traumatic events. Various therapeutic approaches such as tympanoplasty, autologous graft, or application of bone cement are discussed. We report the case of a 46-year-old female patient who developed acute hearing loss in her left ear after finger manipulation. Clinical evaluation revealed axial displacement of the handle of the malleus and audiometry indicated conductive hearing loss. After otoscopy, audiometry, and computed tomography, tympanoscopy was indicated due to suspicion of ossicular chain disruption. Intraoperatively, an isolated fracture of the handle of malleus was found, which was treated with glass ionomer cement. Following postoperative examination, there was progressive improvement in the acoustic transmission component, such that a normal hearing threshold was observed 4 months postoperatively. This case report underlines the importance of precise diagnosis and individualized treatment for rare middle ear injuries.

Characterization of hearing loss in pediatric patients with osteogenesis imperfecta.

INTRODUCTION: Osteogenesis imperfecta (OI) is a common heritable disorder affecting type 1 collagen. The sequelae of OI vary, but hearing loss is a significant complication with 46-58 % of patients having some degree of hearing loss. Previous studies have suggested patients with OI may have conductive, sensorineural, or mixed hearing loss. Majority of these studies focus on the adult population. OBJECTIVES: Identify a relationship between OI and hearing loss in the pediatric population. METHODS: The TriNetx Analytics Network, a federated health research network that aggregates the de-identified electronic health record data of over 78 million patients across the United States, was queried for patients 18 years old or younger with a diagnosis of OI. Patients in this group with diagnosis of sensorineural, conductive, or mixed hearing loss were recorded. Patients with diagnoses of congenital cytomegalovirus, congenital inner ear malformations, and noise-induced hearing loss were excluded from analysis. RESULTS: Out of 3256 patients 18 years old or younger with OI, 10.07 % (95 % CI: 9.06-11.16) had a history of any form of hearing loss, 5.71 % (95 % CI: 4.94-6.57) had conductive hearing loss, 3.01 % (95 % CI: 2.45-3.66) had sensorineural hearing loss, and 1.35 % (95 % CI: 0.98-1.81) had mixed hearing loss. Relative risks for diagnosis of any type of hearing loss, conductive hearing loss, sensorineural hearing loss, and mixed hearing loss were calculated: 5.90 (95 % CI 5.32-6.53), 5.08 (95 % CI 4.42-5.84), 6.18 (95 % CI 5.09-7.51), and 13.86 (95 % CI 10.33-18.59) respectively. DISCUSSION: This study is the largest to date that describes a relationship between OI and conductive, sensorineural, and mixed hearing loss. Pediatric patients with OI are almost five times as likely to have any type of hearing loss. There was a significant increased risk in each subgroup, but conductive hearing loss was the most common for hearing loss in children with OI. The highest risk subtype when compared to controls was mixed hearing loss.