Linear transmission between malleus and stapes in cases with incus necrosis.

OBJECTIVE: The outcome of cartilage interposition ossiculoplasty was assessed in cases of incus necrosis after posterior malleus repositioning in the plane of the stapes, in terms of hearing gain after ossicular reconstruction. METHODS: A retrospective observational study was conducted of 30 patients admitted to an Ain Shams University hospital from March 2021 to September 2021. All patients with ossicular disruption due to chronic suppurative otitis media and hearing loss of more than 40 dB were included in the study. Pure tone audiometry was conducted for each patient after three months, six months and one year post operation. RESULTS: The audiogram showed a post-operative air-bone gap of 20 dB or less in 83.33 per cent of patients (n = 25) at three months post-operatively and in 80 per cent of patients after six months; after one year, the results remained the same. CONCLUSION: The use of cartilage interposition after malleus posterior mobilisation represents an excellent partial ossicular replacement technique.

Otosclerosis under the magnifying glass.

Otosclerosis is a bone condition affecting the stapes bone within the otic capsule, and its exact cause is still unknown. It is characterized by a lack of proper remodeling of newly formed vascular and woven bone, leading to the development of abnormal osteons and the formation of sclerotic bone. Bilateral otosclerosis is seen in 80% of patients and 60% of otosclerosis patients have a family history of the condition. The etiology of this disease is still unknown, there are lots of theories to explain it. The histopathological (HP) studies of otosclerosis showed that osteoblasts, osteoclasts, vascular proliferation, fibroblasts, and histiocytes were observed in the stapes footplate. The onset of the symptoms occurs by the early third decade of life, usually it doesn't start later. In otosclerosis, the energy exerted by sound at the level of the tympanic membrane is reduced in the inner ear due to the fixation and rigidity of the ossicular chain, leading to hearing loss, especially for low frequencies. The primary clinical symptom of otosclerosis is conductive hearing loss but it is important to note that sensorineural hearing loss and mixed hearing loss can also occur as secondary symptoms of the condition. Another symptom present in patients with otosclerosis is tinnitus. The paper carried out a retrospective study of 70 patients diagnosed with otosclerosis in the Department of Otorhinolaryngology of Emergency City Hospital, Timisoara, Romania, between January 2021 to December 2022. Tissue fragments were processed at Service of Pathology by standard Hematoxylin-Eosin staining. The HP diagnosis was completed using Masson's trichrome staining, Giemsa histochemical staining, and immunohistochemical (IHC) reactions with anti-cluster of differentiation (CD)20, anti-CD3, anti-CD4, anti-CD8, anti-CD34, and anti-CD31 antibodies. The microscopic examination showed a chronic diffuse inflammatory infiltrate that consisted predominantly of mature T-lymphocytes, immunohistochemically positive for CD3, CD4 and CD8. There were also present rare CD20-positive B-lymphocytes. Among the lymphocytes, relatively numerous mast cells were identified, highlighted histochemically by the Giemsa staining. They had numerous purple-violet intracytoplasmic granules. In the connective tissue support, a relatively rich vascular network was identified, consisting of hyperemic capillaries, highlighted immunohistochemically with anti-CD31 and anti-CD34 antibodies. Bone tissues trabeculae showed extensive areas of fibrosis. The collagen fibers were highlighted by Masson's trichrome staining, being stained in green, blue, or bluish green.

Genetic variants and altered expression of SERPINF1 confer disease susceptibility in patients with otosclerosis.

Otosclerosis (OTSC) is a focal and diffuse bone disorder of the human middle ear characterized by abnormal bone growth and deposition at the stapes' footplate. This hinders the transmission of acoustic waves to the inner ear leading to subsequent conductive hearing loss. The plausible convections for the disease are genetic and environmental factors with yet an unraveled root cause. Recently, exome sequencing of European individuals with OTSC revealed rare pathogenic variants in the Serpin Peptidase Inhibitor, Clade F (SERPINF1) gene. Here, we sought to investigate the causal variants of SERPINF1 in the Indian population. The gene and protein expression was also evaluated in otosclerotic stapes to ameliorate our understanding of the potential effect of this gene in OTSC. A total of 230 OTSC patients and 230 healthy controls were genotyped by single-strand conformational polymorphism and Sanger sequencing methods. By comparing the case controls, we identified five rare variants (c.72 C > T, c.151 G > A, c.242 C > G, c.823 A > T, and c.826 T > A) only in patients. Four variants c.390 T > C (p = 0.048), c.440-39 C > T (p = 0.007), c.643 + 9 G > A (p = 0.035), and c.643 + 82 T > C (p = 0.005) were found to be significantly associated with the disease. Down-regulation of SERPINF1 transcript level in otosclerotic stapes was quantified by qRT-PCR, ddPCR and further validated by in situ hybridization. Similarly, reduced protein expression was observed by immunohistochemistry and immunofluorescence in otosclerotic stapes that corroborate with immunoblotting of patients' plasma samples. Our findings identified that SERPINF1 variants are associated with the disease. Furthermore, reduced expression of SERPINF1 in otosclerotic stapes might contribute to OTSC pathophysiology.

Cellular voids in the pathogenesis of otosclerosis.

BACKGROUND: Otosclerosis is a common ear disease that causes fixation of the stapes and conductive hearing impairment. However, the pathogenesis of otosclerosis is still unknown. Otosclerosis could be associated with the unique bony environment found in the otic capsule. Normal bone remodelling is almost completely absent around the inner ear after birth allowing degenerative changes and dead osteocytes to accumulate. High levels of inner ear anti resorptive osteoprotegerin (OPG) is most likely responsible for this capsular configuration. Studies have demonstrated how osteocyte lifespan variation creates occasional clusters of dead osteocytes, so-called cellular voids, at otosclerotic predilection sites in the human otic capsule. These cellular voids have been suggested as possible starting points of otosclerosis. AIM: To describe the cellular viability in otosclerotic lesions and compare it to that of cellular voids. MATERIALS AND METHODS: The study was based on unbiased stereological quantifications in undecalcified human temporal bones with otosclerosis. RESULTS: Osteocyte viability was found to vary within the otosclerotic lesions. Furthermore, the results presented here illustrate that inactive otosclerotic lesions consist of mainly dead interstitial bone, much like cellular voids. CONCLUSIONS AND SIGNIFICANCE: Focal degeneration in the otic capsule may play an important role in the pathogenesis of otosclerosis.

The impact of tympanic membrane perforations on middle ear transfer function.

PURPOSE: Injury or inflammation of the middle ear often results in the persistent tympanic membrane (TM) perforations, leading to conductive hearing loss (HL). However, in some cases the magnitude of HL exceeds that attributable by the TM perforation alone. The aim of the study is to better understand the effects of location and size of TM perforations on the sound transmission properties of the middle ear. METHODS: The middle ear transfer functions (METF) of six human temporal bones (TB) were compared before and after perforating the TM at different locations (anterior or posterior lower quadrant) and to different degrees (1 mm, » of the TM, ½ of the TM, and full ablation). The sound-induced velocity of the stapes footplate was measured using single-point laser-Doppler-vibrometry (LDV). The METF were correlated with a Finite Element (FE) model of the middle ear, in which similar alterations were simulated. RESULTS: The measured and calculated METF showed frequency and perforation size dependent losses at all perforation locations. Starting at low frequencies, the loss expanded to higher frequencies with increased perforation size. In direct comparison, posterior TM perforations affected the transmission properties to a larger degree than anterior perforations. The asymmetry of the TM causes the malleus-incus complex to rotate and results in larger deflections in the posterior TM quadrants than in the anterior TM quadrants. Simulations in the FE model with a sealed cavity show that small perforations lead to a decrease in TM rigidity and thus to an increase in oscillation amplitude of the TM mainly above 1 kHz. CONCLUSION: Size and location of TM perforations have a characteristic influence on the METF. The correlation of the experimental LDV measurements with an FE model contributes to a better understanding of the pathologic mechanisms of middle-ear diseases. If small perforations with significant HL are observed in daily clinical practice, additional middle ear pathologies should be considered. Further investigations on the loss of TM pretension due to perforations may be informative.

The aetiology of ossicular chain defects in congenital cholesteatoma.

OBJECTIVE: The aim of the present study was to perform a retrospective review of the lesion sites in congenital middle-ear cholesteatoma and any accompanying ossicular defects, as well as to explore the possible aetiology of congenital middle-ear cholesteatoma associated with such ossicular chain defects. METHOD: The clinical characteristics and pathogenic mechanisms of ossicular chain defects were investigated in 10 patients with early-stage congenital middle-ear cholesteatoma confirmed by surgery, from August 2011 to February 2019. RESULTS: Monofocal cholesteatoma was located in the anterosuperior quadrant in 3 cases and in the posterosuperior quadrant in 7 cases; all 10 cases showed an absence of the long crus of incus, and 8 cases showed a complete or partial absence of stapes superstructure. The lesions were confined to the vestibular window and the stapes region and had no contact with the long crus of the incus or stapes in nine cases. None of the 10 patients had any recurrence of cholesteatoma. CONCLUSION: Although cholesteatoma can cause erosion of ossicles, the present cases suggest that residual epithelium of the cholesteatoma may coexist with ossicular malformations. Therefore, the aetiology of the clinical characteristics in these patients may derive from residual epithelial hinderance of ossicle development.

The Stapes in Otosclerosis: Osteoarthritis of an Ear Ossicle.

Otosclerosis is a pagetoid proliferation of bone remodeling, vascular proliferation, bone resorption and new bone formation in the tympanic region of the temporal bone. The resulting anklyosis of the stapes footplate as it articulates with the oval window is the most common cause of conductive hearing loss in young to middle aged, predominantly Caucasian individuals. The characteristic histologic features have been well documented by autopsy studies of the temporal bone. Although stapedectomy is the surgical treatment for otosclerosis, the stapes specimen may be submitted for gross examination only or not examined at all. A retrospective study of 73 stapedectomy specimens (2008-2019) not including the stapes footplate. Clinical features from the electronic medical record as well as standard histologic sections from surgical specimens were reviewed. Neither the stapedal head nor crura showed histologic features of otosclerosis. There was mild osteoarthritis affecting the head, possibly as a consequence of persistent ossicular vibration superimposed on the ankylosed rigidity. The most common changes were surface fissuring (65%), cartilaginous erosion (49%) and irregularity of the osteochondral interface (51%). An occasional osteophyte (8%) was observed. The ear ossicles, embryologically analogous to long bones of the extremities, develop via endochondral ossification and exhibit articular surfaces of hyaline cartilage. The present observations suggest that a consequence of otosclerotic ankylosis is osteoarthritis of the stapedal head. In this study, the histological features could not be correlated with the severity of hearing loss or duration of clinical disease.

Evaluation of the Presence of Sensorineural Hearing Loss and the Relationship With Intraoperative Findings in Cholesteatoma.

We investigated whether cholesteatoma is associated with sensorineural hearing loss (SNHL) and the effects of a lateral semicircular canal (LSCC) fistula, destruction of stapes, localization of cholesteatoma, and air-bone gap (ABG) size on SNHL. The charts of 159 patients who had received surgery for unilateral cholesteatoma were examined retrospectively. In all patients, air conduction and bone conduction (BC) thresholds in both ears were measured at 500, 1000, 2000, and 4000 Hz. Differences in BC thresholds between ears with cholesteatoma and contralateral ears were calculated. Demographics, localization of cholesteatoma, presence of LSCC fistula, condition of stapes, and ABG size on the affected ear were evaluated. There were significantly greater BC thresholds in ears with cholesteatoma than in normal ears for each frequency. Comparing the average BC differences at the different cholesteatoma locations, there were significant differences between the tympanic cavity + all mastoid cell group and attic, attic + antrum, and tympanic cavity + antrum groups. The BC differences at 4000 Hz were significantly high in patients with LSCC fistula. There were no significant relationships between the condition of the stapes and BC differences at any frequencies. There were significant correlations between average ABG and BC threshold differences at all frequencies. A significant relationship was found between cholesteatoma and SNHL. Patients with advanced cholesteatoma had significantly higher levels of SNHL. The BC threshold differences increased with increases in the ABG.

TORP ossiculoplasty in the presence of an intact stapes superstructure.

INTRODUCTION: In this series, we describe ossicular reconstruction using routine TORP in the presence of the stapes superstructure using a tympanic-membrane-to-footplate configuration. MATERIALS AND METHODS: This is a case series of 12 patients who underwent TORP ossiculoplasties from 1st January 2010 to 31st October 2017. Data collected included demographics, indication for surgery, status of the middle ear and pre-operative and post-operative audiometric data (including pure tone average (PTA) and air-bone gap (ABG)). RESULTS: The mean pre-operative PTA was 56.5 dB and ABG was 39.8 dB. Surgical success (ABG ≤ 20 dB) was achieved in 83.3%, with an average improvement in PTA of 24.3 dB and closure of ABG of 27.1 dB. The mean post-operative ABG was 12.7 dB. CONCLUSION: TORP ossiculoplasty with tympanic-membrane-to-footplate configuration is a feasible means of ossicular reconstruction, independent of the status or spatial arrangement of the remnant ossicles. Using this technique, it is possible to achieve a consistently good outcome for improvement in hearing and closure of ABG.

Genetic and clinical phenotypic analysis of familial stapes sclerosis caused by an NOG mutation.

BACKGROUND: The noggin protein encoded by the NOG gene can interfere with the binding of bone morphogenetic protein to its receptor, thus affecting bone and joint development. The symptoms include abnormal skeletal development and conductive deafness. METHODS: In a retrospective study, clinical data of the proband and her family members, including 8 people and 50 healthy normal controls, were collected. Second-generation sequencing was performed on peripheral blood samples from them. RESULTS: The sequencing analysis indicated that in the proband, the NOG gene had a c.532T > C, p.C178R (cytosine deletion, NM_005450.6:c.532T > C), leading to an amino acid change. The proband's father, grandmother, second sister, and third sister also had this mutation, whereas family members with normal phenotypes did not have the mutation. CONCLUSION: Analysis of this family showed that the novel presentation of the c.532T > C, p.C178R mutation in the NOG gene resulted in syndrome-type autosomal dominant inheritance reflected in a mild clinical phenotype, which is of great importance for further studies of the clinical phenotype and pathogenesis of stapes sclerosis.

Measurement of stapes footplate thickness in otosclerosis by ultra-high-resolution computed tomography.

BACKGROUND: Ultra-high-resolution computed tomography (U-HRCT) utilizes a 1024 × 1024 matrix with 0.25-mm section thickness, offering better spatial resolution than conventional multi-detector row CT to detect anatomic data for otologic surgery. AIMS: We examined stapes footplate thickness using U-HRCT in relation to stapedotomy to predict the difficulty of the surgical procedure. MATERIALS AND METHODS: Subjects were 12 otosclerosis patients and 25 controls who underwent diagnostic U-HRCT. A profile curve (Hounsfield units) was used to measure stapes footplate thickness along a perpendicular line across the stapes footplate in a plane parallel to the lateral semicircular canal. RESULTS: Footplate thickness was smaller at the midpoint than just before the anterior crus and just after the posterior crus. Interobserver variability was lowest at the midpoint, where foot plate thickness was significantly greater in the affected ear in otosclerosis patients compared with controls (0.60 ± 0.09 mm vs 0.46 ± 0.04 mm; p < .001). Otosclerosis patients were detected using U-HRCT with a high area under the curve. Difficulty in the stapes opening procedure correlated with stapes footplate thickness. CONCLUSIONS: Footplate thickness on U-HRCT correlated with temporal bone anatomy and corresponded to surgical difficulty. Significance: U-HRCT-derived anatomic data is useful for evaluating the stapes.

The impact of stapes surgery on osteogenesis imperfecta: a retrospective comparison of operative outcomes with those for patients with otosclerosis.

BACKGROUND: While stapes surgery has long been employed as a means of improving hearing in patients with otosclerosis, it remains controversial as to whether such surgical intervention is of value in patients with osteogenesis imperfecta (OI). AIMS: This study was therefore designed to determine whether stapes surgery could still be reliably performed in OI patients as it is in otosclerosis patients. MATERIALS AND METHODS: Seventeen OI patients and 18 matched otosclerosis patients were included. In both of these groups, 22 ears underwent primary stapes surgery. Audiometric follow-up data for patients in these two treatment groups were available for between 2 weeks and 25 years post-surgery. RESULTS: We detected significant improvements in both the short- and long-term mean air conduction threshold and reduced air-bone gap (ABG) in both treatment groups as measured via postoperative follow-up audiometry. There was no difference between the OI and otosclerosis patient groups with respect to short- and long-term postoperative audiometric. Conclusions and significance: The majority of patients with both OI and otosclerosis that underwent stapes surgery achieved beneficial gains in hearing function. We did not observe any differences in short- or long-term post-operative hearing gains or ABG reductions between the two groups.

Multiple synostoses syndrome: Clinical report and retrospective analysis.

Multiple synostoses syndrome (SYNS1; OMIM# 186500) is a rare autosomal dominant disorder reported in a few cases worldwide. We report a Chinese pedigree characterized by proximal symphalangism, conductive hearing loss, and distinctive facies. We examined the genetic cause and reviewed the literature to discuss the pathogeny, treatment, and prevention of SYNS1. Audiological, ophthalmological, and radiological examinations were evaluated. Whole-exome sequencing (WES) was performed to identify mutations in the proband and her parents. Sanger sequencing was used to verify the results for the proband, parents, and grandmother. The literature on the genotype-phenotype correlation was reviewed. The patient was diagnosed with multiple synostoses syndrome clinically. WES and bioinformatic analysis revealed a novel missense mutation in the NOG gene, c.554C>G (p.Ser185Cys), cosegregated in this family. The literature review showed that the phenotype varies widely, but the typical facies, conductive hearing loss, and proximal symphalangism occurred frequently. All reported mutations are highly conserved in mammals based on conservation analysis, and there are regional hot spots for these mutations. However, no distinct genotype-phenotype correlations have been identified for mutations in NOG in different races. Regular systematic examinations and hearing aids are beneficial for this syndrome. However, the outcomes of otomicrosurgery are not encouraging owing to the regrowth of bone. This study expanded the mutation spectrum of NOG and is the first report of SYNS1 in a Chinese family. Genetic testing is recommended as part of the diagnosis of syndromic deafness. A clinical genetic evaluation is essential to guide prevention, such as preimplantation genetic diagnosis.

[Otosclerosis. 2nd part. Diagnosis]. / Otosclerosis. 2. rész. Diagnózis.

Otosclerosis is a bone remodeling disorder affecting exclusively the human temporal bone which causes small bony lesions in the otic capsule. The symptoms depend on the location and the extent of the otosclerotic foci. Hence, clinically the most relevant sign is the conductive hearing loss due to the stapedial otosclerosis with fixation of the stapes footplate. In many cases, the specific anamnestic features, the age of presentation and usually the absence of tympanic membrane pathology can provide a strong clinical suspicion for otosclerosis. Although audiometric and imaging examinations and VEMP testing can confirm our preoperative diagnosis, the histolopathologic examination of the removed stapes footplate is the most accurate way to determine the diagnosis. Orv Hetil. 2019; 160(51): 2007-2011.

Easy Stapes Subluxation in 22q11.2 Deletion Syndrome: A Clinical Capsule and Literature Review.

OBJECTIVES: This report describes iatrogenic stapes subluxation in a 22q11 deletion syndrome patient and reviews the human and murine literature for evidence that these patients have stapes malformations. We aim to alert otologic surgeons regarding the possibility of stapes footplate abnormalities in 22q11 deletion patients. PATIENT: An adult woman with known 22q11 deletion syndrome. Additionally, the literature review focused on other patients with 22q11 deletion syndrome as well as mouse models of this disorder. INTERVENTIONS: A combination of diagnostic and therapeutic interventions were conducted consisting of middle ear exploration, removal of ossicular chain adhesions, and ultimately ossicular reconstruction. RESULTS: The stapes footplate was poorly attached to the oval window in our patient. During removal of ossicular adhesions, the entire stapes subluxed requiring placement of a stapes prosthesis. The postoperative audiogram was similar to the preoperative audiogram. Literature review identified one other case of stapes subluxation in a patient with 22q11 deletion syndrome, and mouse models suggest that the stapes footplate has an abnormal connection to the oval window in those affected by 22q11 deletion syndrome. CONCLUSIONS: Patients with 22q11 deletion syndrome have chronic middle ear pathology, and if middle ear exploration is undertaken, the surgeon should be aware that the stapes may have a weak attachment to the oval window. This could put the stapes at risk of injury and contribute to conductive hearing loss.

Optical Coherence Tomography Doppler Vibrometry Measurement of Stapes Vibration in Patients With Stapes Fixation and Normal Controls.

HYPOTHESIS: Ears with otosclerotic stapes fixation will exhibit lower-than-normal levels of ossicular mobility as measured by Optical Coherence Tomography Doppler Vibrometry (OCT-DV). BACKGROUND: OCT-DV measures the vibration of middle ear structures in response to sound non-invasively through the intact tympanic membrane. This allows, in particular, direct measurement of the vibration at the lenticular process of the incus which is expected to be lower in patients with otosclerotic stapes fixation. METHODS: OCT-DV was performed on ears presumptively diagnosed with otosclerosis (n = 13) and a group of normal control ears (n = 42). Displacement was measured at the umbo and the lenticular process of the incus in response to 500 and 1000 Hz stimulus tones at 100 dBSPL. MAIN OUTCOME MEASURE: The ability to discriminate between groups was assessed using receiver operator characteristic analysis, with the main outcome measures being the area-under-curve (AUC) and the sensitivity and specificity. RESULTS: For the best condition tested (500 Hz at the incus), the AUC was 0.998 and discriminated the otosclerotic from normal ears with a sensitivity/specificity of 1.00/0.98. One anomalous patient with surgically confirmed stapes fixation exhibited hypermobility at the umbo, possibly due to a partial ossicular discontinuity. Despite the high umbo mobility, this patient's stapes fixation was correctly discriminated based on the measured incus vibration levels. CONCLUSIONS: OCT-DV is a promising tool for preoperatively assessing ossicular mobility non-invasively in the clinic. Our results suggest OCT-DV may also be useful in discriminating other ossicular pathologies that result in conductive hearing loss.

Predictable prosthesis length on a high-resolution CT scan before a stapedotomy.

OBJECTIVE: (1) To assess the correlation between preoperative high-resolution CT (HRCT) imaging measurement from the long process of the incus to the footplate and the length of intraoperative selected prosthesis. (2) To determine if HRCT has a predictive value of prolapsed facial nerve during stapedotomy. MATERIALS AND METHODS: We evaluated in our tertiary care center, in a retrospective case series, 94 patients undergoing primary stapedotomy. Preoperative temporal bone HRCT scans were reformatted in the plane of the stapes to measure on the same section the distance between the long process of the incus and the footplate. Measurement was performed by otolaryngology resident and neurotologist. We analyze the interobserver correlation and the mean length measured on the HRCT to the selected prosthesis size intraoperatively. RESULTS: Mean HRCT measurement of the incus long process/footplate distance assessed by the otolaryngology resident and neurotologist was 4.34 and 4.38 mm, respectively. Interobserver correlation was statistically significant [intraclass correlation coefficient (ICC) of 0.679 (p < 0.001)]. Mean selected prosthesis length intraoperatively was 4.36 mm. Correlation between the mean selected length prosthesis and the mean HRCT measurement was also statistically significant [ICC of 0.791 (p < 0.001)]. Postoperatively, a statistical improvement was shown in air conduction (p < 0.001), bone conduction (p < 0.001) and air-bone gap reduction (p < 0.001). 2 cases of facial nerve covering one-half of the oval window were identified by HRCT and confirmed intraoperatively. CONCLUSION: HRCT is a valuable tool to predict preoperatively the length of the stapedotomy prosthesis. Moreover, it might be helpful to identify a potential prolapsed facial nerve, to confirm the diagnosis of otosclerosis and to rule out other abnormalities. Ultimately, it may optimize the stapedotomy procedure planning.

[The diagnostics and surgical treatment of the patients presenting with otosclerosis in the Primorye Territory]. / Diagnostika i khirurgicheskoe lechenie patsientov s otosklerozom v Primorskom krae.

The objective of the present study was to characterize the effectiveness of the audiological and radiological diagnostic techniques as well as the results of the surgical treatment of the patients presenting with otosclerosis based at the hospital clinic of the Pacific State Medical University. The analysis included 49 surgical interventions for stapedoplasty carried out on 42 patients during the period from 2015 to 2017. The tympanic form of the disorder was diagnosed in 51.02% of the cases, the mixed form I in 16.28%, and the mixed form II in 32.7%. The excellent results within two months after surgery were obtained in 87.2% of the treated patients, the good and satisfactory results in 8.72% and 4.08% of them respectively.

A New Reversal Steps Stapedotomy.

OBJECTIVE: To evaluate whether removal of the stapes head and not the entire stapes superstructure prevents footplate complications. STUDY DESIGN: Prospective study. PATIENTS: One hundred and forty-five patients with otosclerosis were included. Patients were divided into 2 groups as follows: Individuals in group 1 (n = 75) underwent removal of the entire stapes superstructure; individuals in group 2 (n = 70) underwent removal of the stapes head only. These 2 groups were further subdivided into 2 subgroups based on the type of otosclerosis. Group 1a included 55 cases of marginal otosclerosis, while group 1b included 15 diffusive and 5 biscuit otosclerosis cases. Group 2a included 51 marginal otosclerosis cases, while group 2b included 14 diffuse and 5 biscuit otosclerosis cases. Estimations were made regarding statistical correlation between type of reversal steps stapedotomy, otosclerosis type, incidence of footplate complications, and hearing results. RESULTS: There were 9 footplate complications in group 1b and no complications in group 2b. This difference was significant. Air-bone gap closure to within 10 dB was achieved in 65% of cases in group 1b and 94.7% in group 2b. This difference was significant. CONCLUSION: The new stapedotomy prevented footplate complications and improved hearing results in diffuse and biscuit otosclerosis.

Practicality analysis of the staging system proposed by the Japan Otological Society for acquired middle ear cholesteatoma: A multicenter study of 446 surgical cases in Japan.

OBJECTIVE: The Objective of this study was to assess the practicality of the cholesteatoma staging system proposed by the Japan Otological Society (JOS) for acquired middle ear cholesteatoma (the 2010 JOS staging system). METHODS: Between 2009 and 2010, 446 ears with retraction pocket cholesteatoma underwent primary surgery at 6 institutions in Japan. The extent of cholesteatoma was surgically confirmed, and classified into three stages. RESULTS: The cholesteatoma affected the pars flaccida in 325 ears (73%), the pars tensa in 100 ears (22%), and both regions combined in 21 ears (5%). The hearing outcome (postoperative air-bone gaps dB) worsened as follows (Stage I, II, III): 84%, 68%, 53% in pars flaccida; 71%, 62%, 30% in pars tensa, and 42% at Stage II, and 50% at Stage III in the combined group. The incidence of residual cholesteatoma increased as follows (Stage I, II, III): 2%, 12%, 23% in pars flaccida; 7%, 30%, 21% in the pars tensa group. The severity of disease was reflected in postoperative hearing and increasing incidence of recurrence rate. CONCLUSION: The 2010 JOS staging system is suitable for evaluating initial pathology. It is particularly practical for standardizing reporting of retraction pocket cholesteatoma and for adjusting for the severity of the condition during outcome evaluations. It may also provide information that is useful for counseling patients.