Temporal Bone High Resolution Computed Tomography Findings in a Case of Postpartum Otosclerosis.

We describe temporal bone high resolution computed tomography (HRCT) findings in a case of bilateral mixed fenestral and retrofenestral otosclerosis with onset in the postpartum period. This condition should be considered in women complaining of postpartum hearing loss and temporal bone HRCT is a fundamental tool leading to the diagnosis.

Comparison of Short-Term Hearing Outcome in Stapedotomy Using either Vein or Fat (Adipose Tissue) as Sealing Material.

Introduction Otosclerosis is a common cause of conductive hearing loss in the adult population that is caused by fixation of the stapes footplate. Cochlear otosclerosis may also present with sensorineural or mixed hearing loss. Surgery is the definitive treatment of choice and, during the procedure, sealing of the oval window with autologous tissue graft around the stapes prosthesis has been routinely done to improve hearing outcome and to mitigate postoperative complications. Objective To evaluate the efficacy of two different types of autologous tissue (vein or fat) grafts as oval window sealing materials in stapedotomy in improving short-term hearing outcomes. Methods In our study, 70 patients with otosclerosis who underwent primary stapedotomy were included. They were divided into group 1 (vein graft) and group 2 (fat graft) based on the type of sealing material used. All patients were followed-up at the end of 3 months, undergoing an audiometric examination to assess the hearing outcome. Results A total of 80% ( n = 28) of the patients in group1 had an air-bone gap (ABG) closure < 10dB, and, in group 2, 85.7% had an ABG closure < 10 dB; this difference was found to be statistically insignificant. A total of 42.9% ( n = 15) of the patients in group 1 and of 31.4% ( n = 11) in group 2 had a significant improvement in bone conduction, while 14.3% ( n = 5) of the patients in group 1 and 17.1% ( n = 6) in group 2 had worsening of average bone conduction postoperatively, which was found to be statistically insignificant. Conclusion Both vein and fat grafts had comparable effects on hearing outcomes when used as sealing materials in stapedotomy.

Conservative Otosclerosis Treatment With Sodium Fluoride and Other Modern Formulations: A Systematic Review.

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.

Bisphosphonate therapy in otosclerosis: A scoping review.

OBJECTIVE: Otosclerosis, a leading cause of deafness in adults, results from defective bone remodeling of the otic capsule. Bisphosphonates have been used to decrease bone remolding in many diseases, including otosclerosis. This study analyzes whether current literature supports bisphosphonate therapy as an effective treatment for otosclerosis. DESIGN: Scoping review. METHODS: A search was performed in three electronic databases; PubMed, Scopus, and Cochrane Control Trials. Articles were screened independently by two masked reviewers based on prespecified inclusion and exclusion criteria. After unmasking, the two reviewers resolved discrepancies through discussion. RESULTS: From the search, 35 unique articles were identified for analysis. The dates of these publications range from 1982 to 2018. Further title and full-text review identified six articles for inclusion in this review. Three of the studies included are randomized controlled trials (RCT)s, and three are retrospective case reviews. These studies analyzed bisphosphonate therapy regimens, but dose and study length varied, making direct comparisons difficult. Only one RCT study was able to show a statistically significant change between patients treated with bisphosphonates compared to a control group. CONCLUSIONS: The efficacy of bisphosphonates for halting bone remodeling in otosclerosis remains unclear. Reviewing the literature, we found significant variations in experimental design and few studies of high-level evidence. Future RCTs investigating therapies for otosclerosis are needed before a firm conclusion about bisphosphonates efficacy as a pharmacological treatment of otosclerosis.Level of Evidence: 3a.

Case study: cochlear implantation in cochlear otospongiosis.

Objective and importance: Cochlear implantation can be performed successfully in patients with otospongiosis of the temporal bone with the potential for excellent audiological outcomes and high patient satisfaction. The purpose of this case report is to highlight the clinical considerations for implantation in cochlear otospongiosis including the need for careful pre-operative implant device selection, intra-operative surgical challenges such as the presence of hypervascularity and possible cochlear ossification resulting in difficulty in placing the electrode array and the possibility of postoperative facial nerve stimulation.Clinical presentation: A 14-year-old girl with cochlear otospongiosis likely due to osteogenesis imperfecta presented with progressive bilateral profound sensorineural hearing loss underwent successful cochlear implantation despite several challenges.Conclusion: Cochlear implantation in patients with cochlear otospongiosis with profound sensorineural hearing loss potentially may be very successful. Thorough pre-operative radiological evaluation is necessary. Possible intra-operative and post-operative challenges unique to these patients must be kept in mind. Adequate precautions should be taken to optimize the likelihood of complete electrode insertion such as using a depth gauge prior to inserting the electrode array and performing an intra-operative x-ray and / or neural response telemetry to confirm correct electrode placement.

Diagnostic utility of labyrinth capsule bone density in the diagnosis of otosclerosis with high resolution tomography. / Utilidad diagnóstica de la densidad ósea de la cápsula laberíntica en el diagnóstico de la otosclerosis por tomografía de alta resolución.

OBJECTIVE: The aim of this study is to compare the bone density around the otic capsule in otosclerotic patients with a control group, and find the cut-off values of bone density from which we can diagnose the disease. MATERIAL AND METHODS: A retrospective case-control study was performed. Bone densities in Hounsfield units (HU) from 28 otosclerotic ears were compared to the densities of 33 non otosclerotic capsules. These densities were measured in eight regions of interest (ROI) where the otosclerotic foci are usually found. The mean density of these regions (PROMED) was taken. Furthermore, the ROC curves of each ROI and the mean density (PROMED) were calculated. RESULTS: All radiological densities in HU of each ROI and the mean density in otosclerotic patients were lower compared to non otosclerotic ears. The area under the ROC curve of each ROI and the mean density showed that the areas with greater accuracy for the diagnosis of otosclerosis were mean density, the fissula ante fenestram, and precochlear region, with cut-off values of 1980HU, 1750HU and 2114HU, respectively. CONCLUSION: The mean density of the otic capsule (PROMED), the density in the fissula ante fenestram (ROI1) and in the precochlear region (ROI3) seem to be the most useful parameters to make a diagnosis of otosclerosis.

Comparison of Short-Term Hearing Outcome in Stapedotomy Using either Vein or Fat (Adipose Tissue) as Sealing Material

Abstract Introduction Otosclerosis is a common cause of conductive hearing loss in the adult population that is caused by fixation of the stapes footplate. Cochlear otosclerosis may also present with sensorineural or mixed hearing loss. Surgery is the definitive treatment of choice and, during the procedure, sealing of the oval window with autologous tissue graft around the stapes prosthesis has been routinely done to improve hearing outcome and to mitigate postoperative complications. Objectives To evaluate the efficacy of two different types of autologous tissue (vein or fat) grafts as oval window sealing materials in stapedotomy in improving short-term hearing outcomes. Methods In our study, 70 patients with otosclerosis who underwent primary stapedotomy were included. They were divided into group 1 (vein graft) and group 2 (fat graft) based on the type of sealing material used. All patients were followed-up at the end of 3 months, undergoing an audiometric examination to assess the hearing outcome. Results A total of 80% (n = 28) of the patients in group1 had an air-bone gap (ABG) closure < 10dB, and, in group 2, 85.7% had an ABG closure < 10 dB; this difference was found to be statistically insignificant. A total of 42.9% (n = 15) of the patients in group 1 and of 31.4% (n = 11) in group 2 had a significant improvement in bone conduction, while 14.3% (n = 5) of the patients in group 1 and 17.1% (n = 6) in group 2 had worsening of average bone conduction postoperatively, which was found to be statistically insignificant. Conclusions Both vein and fat grafts had comparable effects on hearing outcomes when used as sealing materials in stapedotomy.

Medical Management of Otosclerosis.

Otosclerosis/otospongiosis is a primary osteodystrophy of the otic capsule that affects genetically predisposed individuals and leads to progressive hearing loss. Diagnosis is usually clinical, based on the findings of anamnesis, physical examination, and audiometric evaluation. However, high-resolution computed tomography scan and MRI have played an important role in the diagnosis and therapeutic approach of otosclerosis and in assisting in the differential diagnosis. The therapeutic approach is aimed at preventing, or at least minimizing, disease progression while attempting to restore hearing. The use of sodium fluoride and bisphosphonates can be an important adjunct, perhaps even primary treatment, in managing active lesions.

Diagnostic performance of high resolution computed tomography in otosclerosis.

AIM: To determine the sensitivity and specificity of high resolution computed tomography (HRCT) in the diagnosis of otosclerosis. METHODS: A systematic literature review was undertaken to include Level I-III studies (Oxford Centre for Evidenced based Medicine) that utilised HRCT to detect histology confirmed otosclerosis. Quantitative synthesis was then performed. RESULTS: Based on available level III literature, HRCT has a relatively low sensitivity of 58% (95%CI: 49.4-66.9), a high specificity, 95% (95%CI: 89.9-98.0) and a positive predictive value of 92% (95%CI: 84.1-95.8). HRCT is better at diagnosing the more prevalent fenestral form of otosclerosis but remains vulnerable to inframillimetre, retrofenestral and dense sclerotic lesions, despite the advent of more advanced CT scanners with improved collimation. CONCLUSION: Whilst the diagnosis of otosclerosis remains largely clinical, HRCT remains the gold standard imaging of choice for the middle ear and serves as a useful adjunct to the clinician, helping to delineate extent of disease and exclude other causes.

Caracterización de pacientes con otosclerosis con estapedotomia a través de abordaje trascanal y retroauricular en un hospital universitario / Characterization of patients with otosclerosis with transcanal and retroauricular stapedotomy in a university hospital

Introducción: la otosclerosis manejada quirúrgicamente con estapedotomía ha mostrado mejora del umbral auditivo mediante un abordaje transcanal o retroauricular. Objetivo: caracterizar la respuesta al tratamiento quirúrgico con estapedotomía transcanal y retroauricular en pacientes con diagnóstico de otosclerosis en un centro universitario. Diseño: estudio observacional descriptivo retrospectivo tipo serie de casos. Metodología: se incluyeron pacientes con diagnósticos de otosclerosis atendidos en el Hospital Universitario Clínica San Rafael quienes cumplieron criterios de elegibilidad entre 2014 y 2020. Resultados: Se incluyeron 33 pacientes intervenidos con abordaje transcanal y 8 con abordaje retroauricular, la edad promedio fue de 46,6 años. La mayoría de pacientes fue de sexo femenino (56,1 %). Se evidenció una mejora del umbral auditivo en el 89,9 % del total de la muestra. La presencia de variantes anatómicas se describió en un 26,8 %, la variante más común fue el prolapso del nervio cuerda del tímpano (14,5 %). La complicación intraoperatoria más común fue la sección del nervio cuerda del tímpano (9,8 %). La presencia de complicaciones y variantes anatómicas fue más prevalente con abordaje retroauricular. Conclusiones: la estapedotomía con abordaje retroauricular y transcanal mostró mejoría del umbral auditivo equiparable a la literatura mundial, la presencia de variantes anatómicas se correlacionó con complicaciones posoperatorias.

Introduction: Otosclerosis managed surgically with stapedotomy has shown improvement in hearing threshold using a transcanal or retroauricular approach. Objective: To characterize the response to surgical treatment with transcanal and retroauricular stapedotomy in patients diagnosed with otosclerosis in a university center. Design: Retrospective descriptive observational study, case series type. Methodology: Patients with a diagnosis of otosclerosis treated at the Hospital Universitario Clínica San Rafael who met the eligibility criteria between 2014 and 2020 were included. Results: 33 patients operated with transcanal approach and 8 with retroauricular approach were included, the average age was 46.6 years. Most patients were female (56.1%). Hearing threshold improvement was evidenced in 89.9% of the total sample. The presence of anatomical variants was described in 26.8%, the most common variant was the prolapse of the chorda tympani nerve (14.5%). The most common intraoperative complication was eardrum cord nerve section (9.8%). The presence of complications and anatomic variants was more prevalent with retroauricular approach. Conclusion: Stapedotomy with retroauricular and transcanal approach showed improvement of hearing threshold comparable to the world literature, the presence of anatomical variants was correlated with postoperative complications.

Magnetic resonance imaging in the evaluation of clinical treatment of otospongiosis: a pilot study.

OBJECTIVES: To evaluate the applicability of magnetic resonance imaging (MRI) as a method for monitoring the activity of otospongiotic lesions before and after clinical treatment. STUDY DESIGN: Prospective, randomized, controlled, double-blind study. SETTING: One single tertiary care institution in a large, cosmopolitan city. METHODS: Twenty-six patients (n = 42 ears) with clinical, audiometric, and tomographic diagnosis of otosclerosis were enrolled. If computed tomography (CT) demonstrated active lesions, these patients underwent MRI to detect otospongiotic foci, seen as areas of gadolinium enhancement. Patients were divided into 3 groups and received treatment with placebo, sodium alendronate, or sodium fluoride for 6 months. After this period, clinical and audiometric evaluations and a second MRI were performed. Each MRI was evaluated by both a neuroradiologist and an otolaryngologist in a subjective (visual) and objective (using specific eFilm Workstation software) manner. RESULTS: Otospongiosis was most predominantly identified in the region anterior to the oval window, and this site was reliable for comparing pre- and posttreatment scans. The patients in the alendronate and sodium fluoride groups had MRI findings that suggested a decrease in activity of otospongiotic lesions, more relevant in the alendronate group. These findings were statistically significant for both subjective and objective MRI evaluations. CONCLUSIONS: MRI shows higher sensitivity than clinical or audiometric assessment for detecting reduction in activity of otospongiosis. The objective MRI evaluation based on software analysis was the most accurate method of monitoring clinical treatment response in otospongiosis.

Tomographic diagnosis and relevant aspects of otosclerosis / Diagnóstico tomográfico e aspectos relevantes da otosclerose

A literature review and pictorial essay were developed to discuss the importance of knowing the main findings and locations of otosclerosis at multidetector computed tomography (MDCT). The authors performed a retrospective review of cases of otosclerosis diagnosed in their institution by means of high resolution multidetector computed tomography. Otosclerosis corresponds to otic capsule dysplasia characterized by metabolic derangement of its endochondral layer. Such condition constitutes a relevant cause of sensorineural hearing loss, affecting about 7% to 10% of the general population. The diagnosis is usually clinical, but imaging methods play a significant role in the anatomical detailing, differential diagnosis, surgical planning and evaluation of postoperative complications. Among such methods, the relevance of MDCT is highlighted. Radiologists should be familiar with the MDCT findings of otosclerosis, as well as with the temporal bone anatomy to assist in the appropriate clinical management of this disease.

Revisão da literatura e ensaio iconográfico foram realizados com o objetivo de discutir a importância do conhecimento das principais características e localizações da otosclerose à tomografia computadorizada multidetectores (TCMD). Foi feita avaliação retrospectiva de casos de otosclerose diagnosticados em nosso serviço, em equipamento multidetectores com técnica de alta resolução. A otosclerose é uma displasia da cápsula ótica, caracterizada pelo desarranjo metabólico de sua camada endocondral. É uma importante causa de perda auditiva neurossensorial, com incidência de cerca de 7% a 10% da população geral. O diagnóstico usualmente é clínico, porém os métodos de imagens são de grande valia para o detalhamento anatômico, diagnóstico diferencial, planejamento cirúrgico e avaliação de complicações pósoperatórias. Dentre esses métodos, deve-se ressaltar o importante papel da TCMD. Os radiologistas devem estar familiarizados com as características da otosclerose, assim como com a anatomia do osso temporal à TCMD, para auxiliar no manejo clínico adequado desta doença.

Mastoid pneumatization and otosderosis-is there a correlation.

The environment versus the genetic influence on the size of the mastoid pneumatization has been debated for years. A concrete evidence of the influence of environmental factors (e.g. inflammation) or heredity on pneumatization is still lacking. In this study the extent to mastoid peumatization in thirty otosclerotic ears was compared with control ears. The size of mastoid pneumatization was measured by the use of Law lateral x-ray projection with the help of planimetry. It was found that the average pneumatized area in otosclerotic ears was 14.73+ -3.55 cm2 compared to 10.60+3.62 cm2 in control ears which was statistically significant. In addition the mean area of mastoid air system was found to be more in the operated ears compared to the contralateral ears, the difference being statistically significant. However there was no correlation of mastoid pneumatization with degree and duration of hearing loss and type of otosclerotic focus. Our findings therefore show a link between otosclerosis and highly pneumatized mastoids thus indicating a likelihood of heredity playing a role in determining final type ot pneumatization.

Evaluation of vestibular functions in otosclerosis before and after small fenestra stapedotomy.

Vestibular functions in twenty-five patients with otosclerosis were analyzed before and after undergoing stapes surgery. Vertigo was present preoperatively in 6 patients, which disappeared completely after 4-6 weeks of surgery in all the patients indicating the usefulness of stapedectomy. Patients with otosclerosis demonstrated hypoexcitability compared to controls, which was statistically significant for right cold and hot irrigation. Our results showed equal culoric abnormalities in patients with mixed hearing loss and conductive loss. Patients with obliterative focus at surgery had impaired vestibular functions compared to non-obliterative foci without any statistical significance. A longer piston used at surgery tends to produce more vestibular damage compared to a smaller piston. Finally, the degree of vestibular dysfunction seems to be inversely proportional to the hearing result.

Otosclerose infantil: relato de caso e revisão da literatura / Pediatric otosclerosis: case report and literature review

A otospongiose é uma osteodistrofia do osso temporal caracterizada pela reabsorção e neoformação óssea desordenada. Clinicamente, caracteriza-se por disacusia condutiva, neurossensorial, e/ou mista, progressiva e zumbidos. O início dos sintomas ocorre entre 30 e 40 anos de idade sendo rara sua manifestação na infância. Descrevemos o caso de um paciente de 11 anos de idade, com quadro de hipoacusia unilateral progressiva há 5 anos. O exame otorrinolaringológico revelou mancha rubra de Schwartze em orelha esquerda. A audiometria, imitanciometria e a tomografia computadorizada demonstraram características sugestivas de otospongiose. Realizamos uma revisão dos aspectos clínicos, diagnósticos e da conduta terapêutica da otospongiose na infância.

Otospongiosis is an osteodystrophy of the temporal bone, characterized by disordered neoformation and deposition of bone, characterized by the presence of a progressive conductive, sensorineural or mixed hearing loss and tinnitus. Typically, otospongiosis presents as a slowly progressive conductive hearing loss in the third to fourth decade of life. Uncommonly children and adolescents may also have conductive or sensorineural hearing loss caused by otosclerosis. We describe a case of an 11-year-old patient, with progressive unilateral conductive hearing loss for 5 years. The otoscopic examination revealed a positive Schwartz's sign in the left ear. Audiometry, impedanciometry and CT scan showed characteristics that suggested otospongiosis. We reviewed clinical aspects, diagnosis and the therapeutic approach for otospongiosis in children.