Objective: To analyze the etiology, diagnosis, and treatment of unexplained conductive hearing loss (UCHL) with intact tympanic membrane. Methods: A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 642 articles were retrieved from databases such as PubMed, Embase, Web of Science, and Cochrane. Fifty-four research articles and 21 case reports were screened out according to the inclusion and exclusion criteria for analysis of the etiology of UCHL. Seven research articles with UCHL who underwent exploratory tympanotomy were selected for data extraction and analysis of clinical characteristics. Results: UCHL is a common manifestation of various diseases, including congenital ossicular anomalies (COA), otosclerosis (OTS), congenital middle ear cholesteatoma (CMEC), oval window atresia, superior semicircular-canal dehiscence, congenital stapedial footplate fixation, middle ear osteoma or adenoma, congenital ossification of stapedial tendon, and so on. A total of 522 patients were included in the 7 articles; among whom OTS showed a tendency to increase with age. The main symptoms were hearing loss, followed by tinnitus, dizziness, ear fullness, ear pain, facial paralysis. A total of 87.5% to 93.0% patients with COA manifested as nonprogressive deafness that occurred since childhood, with tinnitus incidence of 15.6% to 30.2%, and 86.4% to 96.4% patients with OTS presented with progressive hearing loss, with tinnitus incidence of 60.1% to 90.9%. The diagnosis positive rate of high-resolution computed tomography (HRCT) was 33.8% to 87.1%, and CMEC was higher than that of COA (83.3%-100% vs 28.6%-64%). All the articles reported good hearing recovery. The most common surgical complications included taste abnormalities, tinnitus, and dizziness. Conclusion: UCHL presents with similar clinical manifestations and poses challenges in preoperative diagnosis. Exploratory tympanotomy is the primary method for diagnosis and treatment, with good prognosis after removing the lesion and reconstructing hearing during the operation. Children can also safely undergo the surgery.

OBJECTIVE: Revision stapes surgery is a challenging procedure performed in relatively small numbers compared to other middle ear procedures. Despite numerous data on hearing results of different middle ear surgeries, the audiological standards for successful outcome of this procedure are still not clarified. On the basis of well-documented data, we wanted to determine what the expected audiological results and complications are after revision stapes surgery in order to set a realistic threshold for surgical success.
METHODS: After the protocol registration in the PROSPERO database, a systematic review was performed in multiple databases (PubMed, Cochrane, Web of Science, Scopus, ScienceOpen, ClinicalTrials.gov, Google Scholar) according to PRISMA guidelines. Twelve articles were reviewed according to the inclusion criteria. A total of 1032 cases were obtained for evaluation. A modified version of Newcastle-Ottawa Scale (NOS) was used to assess publication quality.
RESULTS: Average air-bone gap (ABG) gain was 17.3 dB, average air conduction (AC) gain was 17.5 dB. The average postoperative air-bone gap was 11.1 dB. The postoperative ABG distribution was the following 0-10 dB: 53.3%, > 10-20 dB: 28.2%, > 20 dB: 18.5%. SNHL as a surgical complication was described in a total of 17 cases (1.6%), no equilibrium disorder was reported.
CONCLUSIONS: The pooled data suggest that revision stapes surgery is an effective solution after failure of previous stapes surgery. However, the results are clearly inferior to those of primary stapedotomies. Hence, we need to apply different expectations and use different standards in the indication and evaluation of this type of surgery.

Introduction  Stapes surgery was traditionally performed with the use of microscopy either through postauricular, endaural or transcanal approaches. Endoscopic stapedectomy ushered a revolution as a new technique with less complications. Objective  To review the outcomes of endoscopic stapes surgery with an emphasis on intraoperative and postoperative clinical and audiological results. Data Synthesis  A literature review on the PubMed, Web of Science, Scopus, the Cochrane Library, and Embase databases was conducted. Endoscopic stapes surgery or stapedotomy were the main keywords used, and we searched for studies and research published from January 2015 to October 2021. Articles on endoscopic stapes surgery were included, and qualitative and descriptive analyses of the studies and outcomes data regarding audiometric changes and postoperative complications were conducted. Articles including patients with cholesteatoma were excluded. A total of 122 studies were retrieved for qualitative and descriptive analyses and to measure the outcomes of endoscopic stapedotomy; only 12 studies met the inclusion criteria, and the rest was excluded. The meta-analysis revealed a statistically significant difference in hearing improvement. The gain in air-bone gap ranged from 9 dB to 16 dB. A low rate of operative and postoperative complications was reported. Conclusions  Endoscopic stapes surgery appears to be a reasonable alternative to microscopic stapes surgery, with shorter operative times, low complication rate, and significant hearing improvement. The endoscopic technique enabled a better visualization and less scutum drilling, which was confirmed by all included studies.

Recidiva di ipoacusia trasmissiva dopo chirurgia della staffa: una narrative review.
Tra le cause di fallimento funzionale della chirurgia della staffa si annovera la recidiva di ipoacusia trasmissiva, che può verificarsi dopo un periodo variabile di miglioramento dell’udito, da giorni/mesi fino a molti anni dopo l’intervento chirurgico. Le cause più comuni di ipoacusia trasmissiva ricorrente includono lo spostamento della protesi, mentre le aderenze fibrose o la ri-obliterazione della platinotomia/finestra ovale dovuta all’otosclerosi, si verificano meno frequentemente. La TC ad alta risoluzione svolge un ruolo importante nell’identificazione della causa della recidiva di ipoacusia trasmissiva. Parallelamente, l’esplorazione intra-operatoria dell’orecchio medio è cruciale per confermare la causa del fallimento funzionale e gestire correttamente il suo trattamento, con la finalità di migliorare l’udito. Nonostante i risultati generalmente peggiori rispetto a quelli della chirurgia primaria, i risultati della revisione di stapedotomia sono nel tempo migliorati grazie ai progressi nella valutazione pre-operatoria, nella visione intra-operatoria, ed al perfezionamento della tecnica chirurgica.
Among the functional failures of stapes surgery is recurrent conductive hearing loss, which can occur after a variable period of hearing improvement, from days/months up to many years after surgery. The most common cause of recurrent conductive hearing loss is prosthesis displacement, while fibrous adhesions or stapedotomy hole/oval window re-obliteration due to otosclerosis, occur less frequently. High resolution computed tomography plays an important role in the identification of the cause of hearing loss recurrence. Parallel to this, intra-operative exploration of the middle ear is crucial to confirm the cause of failure and address its treatment, possibly restoring hearing. While generally worse than after primary surgery, hearing outcomes after revision stapes surgery have improved thanks to advancements in pre-operative assessment, intra-operative view and technical refinements.

Otosclerosis is a pathological condition affecting the temporal bone, and is characterized by remodelling of the labyrinthine bone tissue through a dynamic process of osteolysis and osteogenesis. This condition progressively leads to hearing loss, tinnitus, and vertigo. Stapedotomy, a surgical procedure involving the removal of the stapes superstructure and its replacement with a prosthesis, is the treatment of choice to improve hearing in individuals with otosclerosis. However, vestibular dysfunction is a significant complication associated with this procedure, which can occur intraoperatively or postoperatively, ranging from the immediate postoperative period to weeks, months, or even years after surgery. This paper aims to provide a comprehensive review of the most important causes of vertigo associated with otosclerosis and stapes surgery with the goal of minimizing the incidence of this complication. Understanding the underlying factors contributing to vertigo in this context is crucial for the prevention and effective management of vertigo in patients undergoing stapedotomy.

Otosclerosis, also known as otospongiosis, is a primary osteodystrophy of the otic capsule of the inner ear and one of the leading causes of deafness in adults. The rationale for medical therapy for otospongiosis is to slow down and eventually stop the phase of bone resorption. Conservative treatments include sodium fluoride (NaF), bisphosphonates, and other modern medicines. A systematic review of the existing and published articles and books until April 2021 has been conducted in Medscape, Google Scholar, PubMed, and other databases using appropriate terms. According to the results of the research, the administration of NaF for a period of at least six months stabilizes hearing thresholds (HTs), improves vestibular symptoms, and delays the worsening of tinnitus. The administration of bisphosphonates for a period of at least six months showed significant percentage differences in the improvement of hearing loss, dizziness, and tinnitus remission. In the already existing double-blind studies that were evaluated, groups of patients treated with bisphosphonates for at least 24 months showed greater stabilization of the mean air and bone conduction thresholds than groups of patients treated with a placebo. The new modern medications have not yet been widely administered clinically to draw useful conclusions, although the test results of some of their use are quite encouraging.

OBJECTIVE: Juvenile Otosclerosis (JO) and Congenital Stapes Footplate Fixation (CSFF) are rare ossicular chain disorders seen in the paediatric population and present with conductive hearing loss. Ongoing controversy exists regarding the role of surgical intervention in JO and CSFF given the poorer hearing outcomes and complications when compared with surgical intervention for adult otosclerosis. The objective of this study is to assess the published data on the surgical outcomes of JO and CSFF in order to guide clinicians and counsel patients on the various medical options for these disease entities.
METHODS: A systematic review of MEDLINE, EMBASE and Cochrane was performed with inclusion criteria of children with JO or CSFF and hearing outcomes following stapes surgery. Studies identified by the search were reviewed and assessed by two independent reviewers in line with the PRISMA guidelines.
RESULTS: 464 articles were initially reviewed and 28 articles met inclusion in the systematic review and meta-analysis. A total of 810 ears (473 and 337 cases of JO and CSFF respectively) underwent stapes surgery. Average age at time of surgery for JO and CSFF was 14.3 and 10.2 years old respectively. The mean pre-operative Air-Bone-Gap (ABG) for JO and CSFF was 31.8 ± 5.2 dB and 39.4 ± 10 dB respectively. Following stapes surgery, the mean post-operative ABG for JO and CSFF was 9.6 ± 6 dB and 19.2 ± 12.5 dB respectively. Surgical success rate (defined as ABG <10 dB) was 81% for JO and 41% for CSFF. Mean ABG gain for JO and CSFF was 24.8 dB (95% CI: 18.6-33.1) and 22.6 dB (95% CI: 18.4-27.8) respectively. The reported number of dead ears was 4/473 (0.8%) for JO and 2/337 (0.6%) for CSFF. 23 cases (2.8%) reported sensorineural hearing loss (SNHL) >10 dB.
CONCLUSIONS: CSFF was associated with poorer hearing outcomes compared to JO, however both entities showed similar improvement in ABG post operatively. Counselling patients and their families on the surgical success rates and complications of JO or CSFF is an important part of the decision making process when deciding between a surgical option or conservative measures such as hearing aids.

To evaluate speech outcomes and facial nerve stimulation (FNS) rates in patients with far advanced otosclerosis (FAO) after cochlear implantation.
A systematic review was performed using standardized methodology of Medline, EMBASE, PubMed, Cochrane, and Web of Science databases. Studies were included if adults with FAO underwent cochlear implantation. Exclusion criteria included concurrent otologic history (e.g., Meniere\'s disease, superior canal dehiscence), non-English-speaking implant users, case reports, abstracts, and letters/commentaries. Bias was assessed using the Newcastle-Ottawa Scale for cohort studies and the National Institute of Health Scale for case series. The primary outcome measure was speech discrimination and the secondary outcomes were rates of partial insertion and FNS.
Twenty-seven studies evaluated cochlear implantation in FAO. Due to the heterogeneity of testing methods, statistical pooling of speech discrimination was not feasible, but qualitative synthesis indicated a positive effect of implantation. Pooled rates of FNS were 18% (95% confidence interval, CI 12%-27%) and the rate of partial insertion was 10% (95% CI 7%-15%).
Cochlear implantation in FAO demonstrates significant gains in speech discrimination scores with higher rates of FNS and partial insertion. Laryngoscope, 133:1288-1296, 2023.

This study aimed to establish hearing outcomes after cochlear implantation in patients with otosclerosis.
We conducted a systematic review and narrative synthesis. Databases searched were as follows: MEDLINE, PubMed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov . No limits were placed on language or year of publication. The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement.
Searches identified 474 abstracts and 180 full texts, with 68 studies meeting the inclusion criteria and reporting outcomes in a minimum of 481 patients with at least 516 implants. Patient-reported outcome measures (PROMs) were reported in five studies involving 51 patients. Intraoperative adverse events/surgical approach details and preoperative radiological assessment were reported in 46 and 38 studies, respectively. The methodological quality of included studies was modest, predominantly consisting of case reports and noncontrolled case series with small numbers of patients. Most studies were Oxford Centre for Evidence Based Medicine grade IV.
Access to good rehabilitation support is essential to achieving the good hearing outcomes and PROMs that can be expected by 12 months after implantation in most cases. There was a significant association between the radiological severity of otosclerosis and an increase in surgical and postoperative complications. Postoperative facial nerve stimulation can occur and may require deactivation of electrodes and subsequent hearing detriment.
Hearing outcomes are typically good, but patients should be counseled on associated surgical complications that may compromise hearing. Modern diagnostic techniques may help to identify potentially difficult cases to aid operative planning and patient counseling. Further work is needed to characterize PROMs in this population.

OBJECTIVE: To compare the efficacy and safety characteristics of different materials used for oval window sealing during stapedotomy.
METHODS: A systematic review was conducted according to the PRISMA guidelines. Published international English literature from January 1, 2000 to December 2021 was screened, checking for studies that compared different materials utilization in patients undergoing stapedotomy surgery for otosclerosis or congenital stapes fixation. Data related to the efficacy and safety of each material were extracted. The primary outcome measure was the air-bone gap (ABG) closure after surgical intervention.
RESULTS: Six studies were included in the metanalysis. Because of the heterogeneity of the treatments adopted, we assessed the use of the fat compared to all other treatments, and the use of the gelfoam compared to all other treatments. In the former analysis (fat vs others) we did not identify differences in ABG closure between the groups (p = 0.74), with a low heterogeneity of the results (I2 = 28.36%; Hedge\'s g = 0.04, 95% CI - 0.19 0.27); similarly, we did not identify differences between the use of gelfoam and other treatments (p = 0.97), with a low heterogeneity of the results (I2 = 28.91%; Hedge\'s g = 0.00, 95% CI - 0.20 0.21).
CONCLUSIONS: Numerous options are available for oval window sealing during stapedotomy, with acceptable safety and effectiveness profiles. Based on the current data, no definitive recommendation can be made regarding the choice of one material over another, and the convenience of sealing over no sealing at all.