Mixed Hearing Loss Research Articles

The Spondylocarpotarsal Synostosis Syndrome Case with Ventricular Septal Defect Caused by a Homozygous Frameshift Variation in FLNB

AbstractSpondylocarpotarsal synostosis syndrome (SCT) (OMIM: 272460), an autosomal recessive disorder, is characterized by block vertebrae, carpal–tarsal synostosis, and disproportionate short stature. The clinical features of SCT include dysmorphic facial traits, cleft palate, hypoplastic tooth enamel, brachydactyly, clinodactyly, limited joint movement, and mixed hearing loss. SCT is associated with biallelic nonsense and frameshift pathogenic variants in the FLNB gene. An 8-year-old patient was referred with suspected skeletal dysplasia. The patient presented with short stature, scoliosis, carpal–tarsal synostosis, and ventricular septal defect (VSD). Clinical exome sequence analysis revealed a homozygous frameshift variation in the FLNB gene (c.219_222dup p.Met75Profs*17). No other pathogenic variations in genes related to VSD were found. This study aims to discuss novel clinical findings related to the genotype–phenotype association. To the best of our knowledge, this is the first reported case of SCT with VSD.

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<sup></sup> System) and passive percutaneous bone-conduction hearing devices (Baha<sup></sup> 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<sup></sup> Connect System unsuitable for some patients. In these cases, the Osia<sup></sup> 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.

A Non-Surgical Wearable Option for Bone Conduction Hearing Implants: A Comparative Study with Conventional Bone Conduction Hearing Aids Mounted on Eyeglasses.

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. 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). 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). 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.

Unilateral versus bilateral stapes surgery: A comparative study on auditory outcome and quality of life

Otosclerosis is a well-known disease which mainly affects middle-aged female patients, causing conductive or mixed hearing loss. Diagnosis is essentially made through a complete audiometric battery testing, while treatment substantially relies on stapes surgery.In this study, we analyzed audiometric and Quality-of-Life data from 33 patients in order to refine the surgical indications (unilateral versus bilateral stapes surgery) and assess the best technique (stapedotomy versus partial stapedectomy) and timing of treatment. The original questionnaire OTOS, in the Italian language, was administered to all patients.In this study, most of the patients were female (18/33), with a mean age of 53 years old. Both surgical techniques appear to be effective with no significant difference in post-operative Air Bone Gap. OTOS questionnaire appeared to be effective in assessing quality of life in patients treated by stapes surgery. Post-operative QoL appeared to be similar in bilaterally and unilaterally operated patients, suggesting that a contralateral intervention after effective surgery should be undertaken only if expressly requested by the patient.

Outcome Evaluation of BAHA Attract System Implantations.

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.

Effect of electromagnetic middle-ear implant simulating sites on the stapes spatial motion: A finite element analysis.

The electromagnetic middle-ear implant (MEI) is a new type of hearing device for addressing sensorineural and mixed hearing loss. The hearing compensation effect of the MEI varies depending on the transducer stimulation sites. This paper investigates the impact of transducer stimulation sites on MEI performance by analyzing stapes spatial motion. Firstly, we constructed a human-ear finite element model based on micro-CT scanning and inverse molding techniques. This model was validated by comparing its predictions of stapes spatial motion and cochlear response with experimental data. Then, stimulation force was applied at four common sites: umbo, incus body, incus long process and stapes to simulate the electromagnetic transducer. Results show that at low and middle frequencies, stapes-stimulating and incus-long-process-stimulating produce similar spatial motion to normal hearing; at high frequencies, incus-body-stimulating produces similar results to normal hearing. The equivalent sound pressure level generated by the stapes piston motion is less sensitive to the stimulation direction than that deduced by the stapes rocking motion.

Head and Neck Airbag Penetrating Injuries.

Airbags have significantly decreased fatalities in frontal vehicle accidents, but their deployment can lead to more severe injuries and rarely death. The authors present a case of a penetrating injury causing tissue loss in the left cheek, an "explosion" of the zygomatic arch, and facial nerve damage, discussing the management in the contest of literature examining similar injuries in the head and neck region. Reviewing English literature from 1995 to 2023, 9 patients experienced severe airbag-related penetrating head and neck injuries, in 3 cases resulting in death. In addition, we describe the case of a young male reporting a deep cheek laceration with facial nerve damage, tissue loss, and comminuted maxilla-zygomatic fracture; 48 hours after trauma, he underwent surgical reconstruction using a bilobed flap, direct suture of the resected masseter muscle, closed reduction of the comminuted zygomatic arch fracture, and direct neurorrhaphy of the lacerated facial nerve. Five months after surgery, facial nerve function recovery was observed. Mouth opening was restored, but a mixed low-medium hearing loss persisted for high frequencies. In another case marginal nerve damage was reported, but management is not described. The use of seat belts and airbags has led to a significant reduction in the incidence of facial fractures and fatalities after motor vehicle accidents, but at the same time, in some cases, complex facial trauma and/or penetrating injury can be observed. In case of suspected or certain facial nerve damage, it is mandatory to perform exploration and eventual reconstruction within 72 hours.

Middle Ear Active Implant Indications, Comparative Audiometric Results from Different Approaches, and Coupling with the Vibrant Soundbridge®: A Single Center Experience over More Than 20 Years.

Middle ear active implants, such as the Vibrant Soundbridge (VSB), offer an alternative to reconstructive surgery and other implantable hearing aid systems for patients with conductive, mixed, or sensorineural hearing loss. The primary objective of this work is to describe the auditory results obtained with VSB in our patient cohort, measuring the auditory gain in terms of average tonal thresholds and spoken word discrimination at 65 dB. Secondly, auditory gain differences between different types of hearing loss, coupling to the ossicular chain compared to round and oval windows, and the impact of open versus more conservative surgical approaches, were analyzed. A cross-sectional observational study, with retrospective data collection, was conducted at a tertiary care center. Clinical and audiometric data pre- and post-implantation were included, from patients who underwent VSB device placement surgery between 2001 and 2024. 55 patients with an average age of 62.58 ± 17.83 years and a slight preference in terms of the female gender (52.72%) were included in the study. The average gain in the PTA for all types of hearing loss was 41.56 ± 22.63 dB, while for sensorineural hearing loss (SNHL) the gain was 31.04 ± 8.80 dB. For mixed-conductive hearing loss (C-MHL) a gain of 42.96 ± 17.70 was achieved, notably, in terms of absolute values, at frequencies of 4000 and 6000 Hz, with gains reaching 49.25 ± 20.26 dB at 4 K and 51.16 ± 17.48 dB at 6 K. In terms of spoken word discrimination, for all types of hearing loss, an improvement of 75.20 ± 10.11% was achieved. However, patients with C-MHL exhibited an approximately 13% higher gain compared to those with SNHL (69.32 ± 24.58% vs. 57.79 ± 15.28%). No significant differences in auditory gain were found between open and closed surgical techniques, nor in the proportion of adverse effects, when comparing one technique with the other. The VSB is effective in improving hearing in patients with mixed, conductive, and sensorineural hearing loss, with significant gains at high frequencies, especially through the round window membrane approach. The choice of surgical technique should consider the patient's anatomical characteristics and specific needs in order to optimize auditory outcomes and minimize postoperative complications.

Risk factors and hearing outcomes in infants and young children in KwaZulu-Natal, South Africa.

Targeted new-born hearing screening, based on high risk factors is recommended in the absence of universal new-born hearing screening in resource-constrained settings. The relevance of risk factors listed in the guidelines of high-income countries and used bylow-middle income countriesremains relatively unknown. Risk factors consistent with the epidemiological profile, evolution of risks and disease burden in these countries need to be considered. This study aimed to profile the frequency of risk factors and their manifestation in hearing outcomesof youngchildren in the KwaZulu-Natal province of South Africa. A chart review ofN= 1433 patients' archival audiology records was conducted, conveniently sampled from a single tertiary hospital (n= 351), a provincial assessment and therapy centre (n= 649), a university clinic (n= 291), and two schools for the deaf (n= 142). Overall, 56% of the participants presented with either a conductive, sensorineural or a mixed hearing loss; 62% of the children had between 1 and 2 risk factors present (Mean [M] = 1.1; standard deviation [s.d.] = 0.98). Admission to neonatal intensive care unit, maternal infections, bacterial and viral infections and chemotherapy, from the Joint Committee on Infant Hearing list of high risk factors were significantly associated with hearing loss (p 0.05). Known non-JCIH risks, emerging risks and other statistically significant contextually relevant risk factors were also noted. Understanding the profile of high risk factors in a given context has implications forprevention, early hearing identification and intervention services.Contribution:Targeted new-born hearing screening needs to be based on risk factors that are contextually relevant. This study is one of the first profiling high risk factors for hearing loss in children in KZN, the province with the second highest population in South Africa.

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

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. A 10year (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. 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 ≤ 10mm (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 ≤ 10mm (fourfold, CI 3.6-3.73). Revision surgery episodes increased with diabetic status (1.2-fold, CI 0.06-0.37) and abutment sizes ≤ 10mm (threefold, 3.303-3.304). 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.

Incidence of Hearing Loss in Patients With Neurofibromatosis Type 1 at a Tertiary Care Pediatric Hospital

BackgroundHearing loss has not been thoroughly investigated as a comorbidity in larger cohorts with neurofibromatosis type 1 (NF1). MethodsAvailable audiometric data were reviewed from patients with NF1 seen at a tertiary pediatric hospital to assess prevalence and risk factors for hearing loss. ResultsOf 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. ConclusionsOur 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.

Assessment of Aminoglycoside-Induced Hearing Loss Risk in the Perinatal Period.

This study aimed to determine the prevalence and heteroplasmy level(s) of MT-RNR1 variants m.1555A > G and m.1494C > T, which are associated with aminoglycoside-induced hearing loss, in a general perinatal population. This study also aimed to characterize the association of these variants and their heteroplasmy levels with hearing loss outcomes with and without aminoglycoside exposure. Droplet digital polymerase chain reaction was performed on 479 maternal DNA samples from a general perinatal biobank at our institution to detect the presence and heteroplasmy levels of MT-RNR1 variants m.1555A > G and m.1494C > T. Testing of paired neonatal specimen(s) was planned for positive maternal tests. A retrospective chart review was performed to characterize the population, identify aminoglycoside exposures, and determine hearing outcomes. All maternal samples tested negative for MT-RNR1 variants m.1555A > G and m.1494C > T. Maternal and neonatal subjects had high rates of aminoglycoside exposure (15.9 and 13.9%, respectively). No subjects with sensorineural or mixed hearing loss had documented aminoglycoside exposure. This study demonstrated that a larger sample size is needed to establish the prevalence of these variants as no subjects tested positive. Determination of variant prevalence in the neonatal population, association of variant heteroplasmy levels with hearing outcomes, and reliability of maternal testing as a surrogate for neonatal testing are important next steps toward universal prenatal or newborn screening. · MT-RNR1 variants are associated with aminoglycoside-induced hearing loss.. · Prevalence of MT-RNR1 variants is uncertain.. · Universal screening for MT-RNR1 variants may be indicated..

Characterization of hearing loss in pediatric patients with osteogenesis imperfecta

IntroductionOsteogenesis 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. ObjectivesIdentify a relationship between OI and hearing loss in the pediatric population. MethodsThe 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. ResultsOut 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. DiscussionThis 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.

Modification of skin incision for Baha Attract System Implantations

<b><br>Introduction:</b> In an era of wide accessibility to various systems for hearing impairment prosthetics, there exists a broad spectrum of surgical techniques facilitating system implantation.</br> <b><br>Aim:</b> The aim of the study is to present the technique of vertical incision in the implantation of the Baha Attract magnetic system as an alternative incision, maintaining a compromise between optimizing the surgical procedure and preserving the functionality and quality of life of the patient.</br> <b><br>Materials and methods:</b> The vertical incision technique is presented based on 5 patients treated between December 2022 and March 2023 diagnosed with conductive or mixed hearing loss.</br> <b><br>Results:</b> There were no adverse consequences or complications resulting from the performed surgical incision. All patients experienced uncomplicated healing. Patients are using sound processor magnets ranging from 4–5 in strength and Baha<sup></sup> 6 Max sound processors weighing 11.5 g.</br> <b><br>Conclusions:</b> The vertical incision technique serves as an alternative to the C-shaped perimeter incision, allowing for the optimization and standardization of the surgical procedure, resulting in a smooth scar formation and maintaining good audiological and aesthetic outcomes.</br>

Evaluation of a super powerful bone-anchored hearing system and its users: A retrospective study.

Although the Baha 5SP has been commercially available for six years, very few studies have been performed on the device's efficacy. The current study aims to evaluate the characteristics and audiological results in patients with severe-to-profound mixed hearing loss fitted with this superpower sound processor. This retrospective evaluation was conducted at a tertiary referral centre where a series of 82 adult patients with severe-to-profound mixed hearing loss were implanted with a percutaneous bone-anchored hearing system and fitted with a superpower sound processor between 2016 and 2019. Patients with incomplete or unreliable audiological data (n = 24) were excluded, resulting in 58 data sets for analysis. The main outcome measures were unaided and aided pure-tone thresholds and aided free-field speech perception in quiet. The median unaided air conduction (AC) threshold averaged across 0.5, 1 and 2 kHz (PTA0.5-2kHz) of all patients was 75 dB hearing loss (HL); the median unaided AC averaged across 1, 2 and 4 kHz (PTA1-4kHz) was 84 dB HL. For bone conduction and direct bone conduction, the median PTA0.5-2kHz was 52 and 47 dB HL, respectively. With the superpower device, the median free-field speech reception threshold was 54 dB sound pressure level (SPL), and the median speech perception score at 65 dB SPL was 80%. At least 75% of the patients reached a maximum phoneme score of 70%. For patients with lower scores, the superpower device still provides a substantial hearing benefit. This makes the superpower device particularly suitable for patients with severe-to-profound mixed hearing loss with a contraindication for conventional hearing aids and/or cochlear implants.

Middle Ear Fibrosis Contributes to Hearing Loss in Patients With Myhre Syndrome.

Myhre syndrome (MS) is a rare genetic condition that presents with multiple genetic anomalies including cleft lip and palate and Eustachian tube dysfunction. These patients are at a high risk for airway scarring from intubation and mucosal inflammation. Hearing loss (conductive or mixed, of varying severity) is a common comorbidity in these patients, the exact etiology of which is still unclear. We present the cases of 2 unrelated children with MS who suffered progressive mixed hearing loss from fibrosis and obliteration of the middle ear spaces. Both patients had multiple sets of ear tubes that demonstrated early extrusion. The older patient underwent bone conduction implantation at age 11 which resulted in dramatic improvement of speech recognition and interactive skills. The other younger patient demonstrates a similar trajectory but has not yet undergone implantation. Otolaryngologists should take a cautious approach to surgery of the eardrum and middle ear to avoid unnecessary induction of fibrosis in this susceptible patient population. These cases highlight a newly described etiology for hearing loss and suggest a benefit to bone conduction implantation.

Automated hearing loss type classification based on pure tone audiometry data

Hearing problems are commonly diagnosed with the use of tonal audiometry, which measures a patient’s hearing threshold in both air and bone conduction at various frequencies. Results of audiometry tests, usually represented graphically in the form of an audiogram, need to be interpreted by a professional audiologist in order to determine the exact type of hearing loss and administer proper treatment. However, the small number of professionals in the field can severely delay proper diagnosis. The presented work proposes a neural network solution for classification of tonal audiometry data. The solution, based on the Bidirectional Long Short-Term Memory architecture, has been devised and evaluated for classifying audiometry results into four classes, representing normal hearing, conductive hearing loss, mixed hearing loss, and sensorineural hearing loss. The network was trained using 15,046 test results analysed and categorised by professional audiologists. The proposed model achieves 99.33% classification accuracy on datasets outside of training. In clinical application, the model allows general practitioners to independently classify tonal audiometry results for patient referral. In addition, the proposed solution provides audiologists and otolaryngologists with access to an AI decision support system that has the potential to reduce their burden, improve diagnostic accuracy, and minimise human error.

Management of Petrous Bone Cholesteatoma: The Gruppo Otologico Experience.

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. 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. Retrospective medical record review. Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated. 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. 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.

Stapedotomy Versus Cochlear Implantation for Far Advanced Otosclerosis: Insights From a Patient With Matched Preoperative and Postoperative Function.

To examine patient preference after stapedotomy versus cochlear implantation in a unique case of a patient with symmetrical profound mixed hearing loss and similar postoperative speech perception improvement. An adult patient with bilateral symmetrical far advanced otosclerosis, with profound mixed hearing loss. Stapedotomy in the left ear, cochlear implantation in the right ear. Performance on behavioral audiometry, and subjective report of hearing and intervention preference. A patient successfully underwent left stapedotomy and subsequent cochlear implantation on the right side, per patient preference. Preoperative audiometric characteristics were similar between ears (pure-tone average [PTA] [R: 114; L: 113 dB]; word recognition score [WRS]: 22%). Postprocedural audiometry demonstrated significant improvement after stapedotomy (PTA: 59 dB, WRS: 75%) and from cochlear implant (PTA: 20 dB, WRS: 60%). The patient subjectively reported a preference for the cochlear implant ear despite having substantial gains from stapedotomy. A nuanced discussion highlighting potentially overlooked benefits of cochlear implants in far advanced otosclerosis is conducted. In comparison with stapedotomy and hearing aids, cochlear implantation generally permits greater access to sound among patients with far advanced otosclerosis. Though the cochlear implant literature mainly focuses on speech perception outcomes, an underappreciated benefit of cochlear implantation is the high likelihood of achieving "normal" sound levels across the audiogram.

Piezoelectric Bone Conduction Hearing Implant: A Case Series of Audiological, Surgical and Patient-Reported Outcomes.

Objective: To examine the surgical, audiological and patient-reported outcomes of the Osia 2 implant. Methods: Data from 14 consecutive subjects undergoing implantation between April 2022 and November 2023 were reviewed. Ten subjects had conductive hearing loss, three had mixed hearing loss and one had single-sided deafness (SSD). Warble tone thresholds, Pure Tone Average (PTA4) and Speech Discrimination Score (SDS) in quiet and in noise were determined unaided and aided. The subjective outcome was determined from two standardized questionnaires: (1) International Outcome Inventory for Hearing Aids (IOI-HA) and (2) Speech, Spatial and Qualities of Hearing Scale 12 (SSQ12b). Results: Unexpected postoperative pain was found in four cases. The warble tone thresholds exhibited a consistent reduction across all frequencies, contributing to a mean decrease of 27 dB in the aided PTA4. SDS demonstrated notable improvements, with a 57.3% increase at 50 dB and a 55.6% increase at 65 dB. In noise, SDS exhibited a 43.9% improvement. The mean IOI-HA Score was 3.8, and the mean overall score for SSQ12b was 6.6, with consistent findings across the subgroups. Conclusions: The Osia device emerges as a promising recommendation for individuals with conductive or mixed hearing loss, possibly also for those with SSD. Its safety and efficacy profile aligns with the broader category of active transcutaneous devices, demonstrating a reduced risk of wound infection compared to percutaneous alternatives. Both audiological assessments and subjective evaluations revealed positive outcomes.