Surgical results of 29 ears with congenital middle ear anomalies; Microscopic vs. endoscopic ear surgery.

OBJECTIVE: Although congenital middle ear anomalies include various types of ossicular anomalies, all of these can be treated by ossiculoplasty or stapes surgery. Transcanal endoscopic ear surgery (TEES) is a minimally invasive surgical method for middle ear disease with an excellent surgical view that has been widely adopted worldwide. To determine the efficacy of TEES for middle ear anomalies, we describe the surgical results and compare the hearing outcomes between patients treated by TEES and microscopic ear surgery (MES). METHODS: A total of 39 ears with congenital middle ear anomalies were treated surgically at the University Hospital of Kochi Medical School between January 2011 and December 2021. In total, 29 ears of 23 patients were included in the study. Demographics, type of anomaly, surgical methods, pre- and postoperative hearing thresholds, and surgical complications were investigated by retrospective chart review. RESULTS: Of the 29 ears, 11 were treated by MES and 18 were treated by TEES. There were no differences in sex, age, preoperative hearing thresholds, or rate of stapes surgery between the two groups. The mean air-bone gap improvement was 20.6 dB in the MES group and 28.8 dB in the TEES group; these values were not significantly different. The median operation time was not significantly different between the MES and TEES groups (125 and 130 min, respectively). The improvements of air conduction in class 1 and 2 (stapes surgery) and class 3 (ossiculoplasty) cases were also not different between the groups. CONCLUSIONS: TEES achieved comparable hearing outcomes to MES without postauricular or endaural incisions. Further, class 1 and 2 anomalies demonstrated hearing improvement similar to class 3 without major complications. Since the surgical field limited around the ossicular chain, coupled with the fact that the middle ear anomaly itself does not exhibit inflammation leading to unfavorable bleeding, TEES is a feasible surgical procedure for all types of congenital ossicular anomalies.

Bilateral Congenital Ossicular Anomalies: Are the Anomalies Symmetric for Both Ears?

OBJECTIVE: To evaluate whether bilateral congenital ossicular anomalies (COAs) differ regarding ossicular anomalies and hearing loss severities between the ears of the individual. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral academic center. METHODS: Between March 2012 and December 2022, 7 consecutive patients (14 ears) with surgically confirmed bilateral COAs were included in the study. Preoperative pure-tone thresholds, COA classification according to the Teunissen and Cremers system, surgical procedures, and postoperative audiometric results were compared between the 2 ears of each patient. RESULTS: The median age of the patients was 11.5 (range: 6-25) years. Both ears of each patient were categorized based on the same classification. Three patients possessed class III COAs and the other 4 had class I COAs. The interaural differences in preoperative bone and air conduction thresholds were within 15 dB for all patients. Differences in postoperative air-bone gaps between ears were not statistically significant. The surgical procedures required for ossicular reconstruction were almost identical for both ears. CONCLUSION: The severity of ossicular abnormalities and hearing loss in patients with bilateral COAs were symmetrical between ears, thereby enabling prediction of the characteristics of the contralateral ear based on the findings observed in 1 ear. These symmetric clinical features can aid surgeons when operating on the contralateral ear.

Transcanal endoscopic ear surgery for management of ossicular malformation: clinical outcomes of 17 cases.

BACKGROUND: In recent years, transcanal endoscopic ear surgery (TEES) is known as an innovative and minimally invasive surgery. AIMS: To clarify the usefulness of TEES for the ossicular malformation, we performed a retrospective study. MATERIAL AND METHODS: We examined cases of ossicular malformation performed using TEES at our hospital between April 2015 and April 2020. RESULTS: The hearing level results were countable for 16 cases. Post-operative hearing levels were significantly improved. Transient nausea, tongue paralysis, and taste disorders were observed; however, no other complications were observed. In 2015-2018, some of the cases required the assistance of a surgical microscope. In contrast, all the cases were performed by TEES after 2019 using a powered device to curve the canal. CONCLUSIONS AND SIGNIFICANCE: TEES requires considerable training, and the sensation of depth is difficult to acquire. However, this less invasive method is also helpful for ossicular malformation cases.

Developmental Disruptions of the Human Stapes.

OBJECTIVE: To evaluate and classify developmental malformations of the human stapes. METHODS: Twenty-five temporal bone specimens from 18 patients with congenital stapes malformations were identified in the Mass Eye and Ear temporal bone collection. Serial sections stained with hematoxylin and eosin were examined by light microscopy and the morphology of the stapes was compared to age-matched controls. RESULTS: Each case of stapes malformation could be classified into one of four malformation types based on our current understanding of the embryologic origin of the subunits of the stapes and timing of development. Twenty-seven percent of stapes malformations had a Type I morphology characterized by a hypoplastic or absent inner footplate and hypoplastic to absent mesoderm footplate or oval window. The crura and capitulum may be absent, monopodal or dysmorphic. Eleven percent expressed a Type II malformation with dysmorphic or monopodal capitulum and crura and a fixed footplate. Twenty-seven percent were of Type III with a dysmorphic or monopodal capitulum and or crura. The footplate, and thereby oval window is present and without fixation. The most common malformation, Type IV, was isolated footplate fixation observed in 33% of cases. CONCLUSIONS: Malformations of the human stapes follow consistent patterns of early or late disruptions of the stapes subunits of mesodermal and/or neural crest origin. While the molecular events, including temporal coordination, that lead to a normally formed stapes are not yet fully understood, the observed patterns of human stapes malformation can be consistently classified into one of four patterns of developmental disruption.

Congenital Anomalies of the Ossicular Chain: Surgical and Audiological Outcomes.

OBJECTIVES: In this study, we aim to analyze audiometric outcomes of middle ear surgery in patients with congenital middle ear anomalies. METHODS: In this single center retrospective cohort study, audiological outcomes were extracted from patient files. Patients with a congenital middle ear anomaly treated surgically in a tertiary referral center between June 2015 and December 2020 were included. Pre- and postoperative short- and long-term audiometric data (at ≥3 and ≥10 months respectively) were compared to analyze hearing outcomes. RESULTS: Eighteen ears (15 patients) were treated surgically with an exploratory tympanotomy. At short term follow up statistically significant improvements in air conduction thresholds and air-bone gaps were found. Hearing improved in 94.4% (17/18) of operated ears. Successful outcome, defined as an air-bone gap closure to within 20 dB after surgery, was reached in 44.4% (8/18). Serviceable hearing (air conduction ≤30 dB) was reached in 55.6% (10/18). Negative outcome (any significant deterioration in hearing) occurred in 1 patient: in this ear otitis media occurred during the postoperative course. At long term follow up, available for 50% of the cohort, hearing remained stable in 5 ears, improved in 1 ear and deteriorated in 3, all of which underwent revision surgery. Sensorineural hearing loss due to surgery, or other complications, were not encountered. CONCLUSION: middle ear surgery was found to be an effective treatment option to improve hearing in this cohort of patients with congenital middle ear anomalies. Surgical goals of obtained gain in air conduction thresholds and serviceable hearing levels were met by most patients without the occurrence of any iatrogenic sensorineural hearing loss.

Endoscopic Ossiculoplasty for the Management of Isolated Congenital Ossicular Chain Malformation: Surgical Results in 16 Ears.

OBJECTIVE: The aim of this study is to describe the clinical characteristics and intraoperative findings and further evaluate the efficacy of endoscopic ossiculoplasty for the management of isolated congenital ossicular chain malformation. METHODS: A retrospective study was performed on 16 ears (15 patients) with the isolated congenital ossicular chain malformation who underwent endoscopic ossiculoplasty in our department from May 2017 to January 2019. Endoscopic exploratory tympanotomy was conducted to check the ossicular chain; at the same time, endoscopic ossiculoplasty was performed depending on intraoperative findings. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps (ABGs) were measured before and after surgery, and the hearing outcome was assessed at 6 months postoperatively. RESULTS: The most common malformations of ossicular chain were the missing of the incus long process and stapes suprastructure. A serial assessment of the hearing status was conducted before and 6 months after surgery. It showed the mean postoperative pure-tone average (PTA) was significantly reduced, and the mean postoperative ABG was obviously closed, respectively (P < .001). The mean PTA gain was 36.3 ± 8.6 dB, and the ABG closure was 35.1 ± 8.3 dB; ABG closure to 20 dB or less and ABG closure to 10 dB or less were achieved in 14 cases (87.5%) and 5 cases (31.3%), respectively. No differences were observed in postoperative hearing outcome between type Ⅲ cases and type Ⅳ cases; however, cases with partial ossicular replacement prosthesis implantation showed a larger hearing gain (P = .049) and a higher proportion of postoperative ABG less than 10 dB (P = .021). No facial palsy and significant sensorineural hearing loss occurred; all patients completed the surgery without the need of canalplasty, and the chorda tympani nerve was preserved in all patients. CONCLUSIONS: This research showed endoscopic surgery was effective in the diagnosis and management of isolated congenital ossicular chain malformation; the endoscopic ossiculoplasty provides an alternative method to manage congenital ossicular chain malformation, with comfortable hearing outcome and the advantage of excellent vision and less invasion.

Comparative study of unilateral conductive hearing loss in patients with an intact tympanic membrane.

BACKGROUND: The ossicular chain status is directly associated with the severity of conductive hearing loss (CHL) when the tympanic membrane (TM) is intact. AIMS/OBJECTIVES: To analyze the relationship between intraoperative findings and audiological data in patients with unilateral CHL and an intact TM. MATERIAL AND METHODS: Forty-three patients who underwent surgery at Xinhua hospital from January 1, 2012, to December 31, 2019, were included and categorized based on their intraoperative findings. Demographic data, intraoperative findings, and preoperative and postoperative audiological results of these patients were analyzed. RESULTS: Patients with ossicular chain disruption had significantly better outcomes than those with ossicular chain fixation (air conduction threshold [ACT]: 26.51 ± 8.82 vs. 44.08 ± 14.41 dB, p < .01; air-bone gap [ABG]: 10.66 ± 6.47 vs. 18.91 ± 9.32 dB, p = .04). Patients with ossicular chain deformities had a higher ACT (56.95 ± 13.81 vs. 44.56 ± 15.44 dB, p = .048) and a larger ABG (43.17 ± 9.94 vs. 33.02 ± 13.41 dB, p = .047) preoperatively than those with cholesteatoma; however, the postoperative outcomes between both patient groups were similar. CONCLUSIONS: Congenital ossicular chain anomalies had similar surgical outcome regardless of the pathology. In acquired cases, ossicular chain disruption had better audiological outcomes than ossicular chain fixation. SIGNIFICANCE: Ossicular chain reconstruction can significantly improve the hearing in patients with UCHL with intact TM.

Numerical analysis of the effects of ossicular chain malformations on bone conduction stimulation.

To assess the effects of ossicular chain malformations on the performance of bone conduction hearing aids, a human ear finite-element model that includes an ear canal, a middle ear, and a spiral cochlea incorporating the third windows was established. This finite element model was built based on micro-computed tomography scanning and reverse modelling techniques, and the reliability of the finite element model was verified by comparison with reported experimental data. Based on this model, two main types of ossicular chain malformations, i.e., the incudostapedial disconnection and the ossicles fixation, were simulated, and their influences on bone conduction were analyzed by comparing the trans-cochlear-partition differential pressures. The results indicate that the incudostapedial disconnection mainly deteriorates the bone conduction response at mid frequencies. The stapes fixation has the largest effect among the ossicles fixation with the bone conduction stimulation, which also mainly decreases the mid-frequency response of the bone conduction, especially at 2 kHz. As the speech intelligibility has the most important frequency range at the range between 1 kHz and 2.5 kHz, the mid-frequency deterioration caused by ossicular chain malformations should be compensated in optimizing the design of the bone conduction hearing aids. For treating patients with the ossicular chain malformations, especially for the patients who suffer from the stapes fixation, the output of bone conduction hearing aids' actuator in the middle frequency band should be improved.

Isolated Incudostapedial Cholesteatomas: Unique Radiologic and Surgical Features.

OBJECTIVES: Congenital cholesteatomas originate from epithelial tissue present within the middle ear in patients with an intact tympanic membrane, no history of otologic surgery, otorrhea, or tympanic membrane perforation. They are diagnosed by a pearl-like lesion on otoscopy and computed tomography (CT) scan showing an expansile soft-tissue mass. We describe a series of patients with no prior otologic history presenting with progressive unilateral conductive hearing loss and normal otoscopy. The CT scans showed ossicular erosion without obvious soft-tissue mass. Surgery confirmed incudostapedial erosion found to be cholesteatoma. In this study, we characterize the clinical course of patients diagnosed with isolated incudostapedial cholesteatoma (IIC) and review possible pathologic mechanisms. METHODS: Retrospective review of IIC cases treated by the Department of Pediatric Otolaryngology, Rady Children's Hospital, San Diego, 2014 to 2020. Data included patient demographics, clinical features, imaging, surgical findings, and audiologic data. RESULTS: Five patients were diagnosed with IIC (3 [60%] female; mean age at presentation 10.7 years [range 5.5-16.0]). All patients presented with postlingual unilateral conductive hearing loss and normal otoscopy without any past otologic history; delay in diagnosis ranged from 4 months to several years. The CT scans showed ossicular chain erosion with an absent long process of the incus and/or stapes superstructure. All patients underwent middle ear exploration, revealing a thin layer of cholesteatoma in the incudostapedial region, confirmed by histopathology. Mean preoperative speech reception threshold was 55 dB and improved to a mean of 31 dB in the 4 patients who underwent ossicular chain reconstruction. CONCLUSION: Isolated incudostapedial cholesteatoma should be included as a possible etiology in pediatric patients with insidious onset of unilateral conductive hearing loss with normal otoscopy, unremarkable otologic history, and a CT scan showing ossicular abnormality/disruption without notable middle ear mass. These patients should be counseled preoperatively regarding the possibility of cholesteatoma and should undergo middle ear exploration with possible ossiculoplasty.

Wideband absorbance pattern in adults with otosclerosis and ossicular chain discontinuity.

OBJECTIVES: Evidence from previous literature had shown that the use of a single frequency probe tone is not sensitive enough to detect middle ear pathologies, especially related to the ossicles, which hinders accurate diagnosis. The goal of the present study was to compare the outcome of wideband absorbance (WBA) tympanometry and to determine the difference in WBA pattern in adults with otosclerosis and ossicular chain discontinuity. MATERIALS AND METHODS: Estimated adult cases of otosclerosis (10 ears) and ossicular chain discontinuity (06 ears) along with healthy individuals (10 ears) in the age range of 24 to 48 years (mean age: 38.6 years) were considered for the study. WBA was measured at peak and ambient pressure along with resonance frequency and compared with the data obtained from the healthy individuals to determine the WBA pattern. RESULTS: Data analysis revealed a distinct WBA pattern showing high absorbance at 750 Hz for ossicular chain discontinuity compared to healthy individuals, whereas the otosclerosis group showed reduced absorbance (p < 0.05) at low frequencies (250 Hz to 1500 Hz). WBA measured at the peak and ambient pressure did not elicit any significant difference across the frequencies. Also, the average WBA tympanogram measured between 375 Hz and 2000 Hz showed a significant difference in ambient pressure only in the otosclerosis group. In comparison to healthy individuals (901 Hz), ossicular chain discontinuity showed a significant reduction in resonance frequency (674 Hz), whereas in cases with otosclerosis had higher resonance frequency (1445 Hz). CONCLUSIONS AND SIGNIFICANCE: The present study showed different WBA patterns between the groups and the absorbance values were significantly different at the low frequencies. This suggests that WBA has the potential to differentiate ossicles related pathologies from normal and also between the ear with otosclerosis and ossicular chain discontinuity.

Availability of audiological evaluation for the differential diagnosis of clinical otosclerosis.

OBJECTIVE: The availability of an audiological evaluation for the differential diagnosis of clinical otosclerosis (OS) was examined. METHODS: Included were 98 patients (107 ears) with OS and 19 patients (20 ears) with incudostapedial disconnection (ISD) diagnosed by surgery between 2009 and 2017 at Aichi University Hospital. Results of preoperative pure-tone audiometry and impedance audiometry were analyzed. The most reliable index for distinguishing the two diseases was evaluated, and the cut-off value, sensitivity, and specificity were calculated. RESULTS: In the univariate analysis, age, static compliance, reversed ipsilateral acoustic reflex (reversed IAR) at 0.5 kHz and 2 kHz, negative contralateral acoustic reflex (negative CAR), difference between the air-bone gap at 0.25 and 2 kHz (0.25-2 kHzABG) and that at 0.25 and 4 kHz (0.25-4 kHzABG) showed statistical significance between the two diseases, whereas the Carhart notch did not. Multivariate logistic regression analysis by the variable selection method showed that reversed IAR (0.5 kHz), negative CAR, and 0.25-4 kHzABG were statistically significantly better factors for the differential diagnosis of the two diseases. The receiver operating characteristic curve for diagnosing OS using the difference between air-bone gap at 0.25 kHz and 4 kHz showed moderate accuracy. If the cut-off value of 0.25-4 kHzABG was 10 dB, then the sensitivity and specificity were 81.1% and 55.0%, respectively. In other words, if the cut-off value of 0.25-4 kHzABG was less than 10 dB, then the possibility of diagnosing ISD becomes higher, and if greater than 10 dB, then the possibility of diagnosing OS becomes higher. CONCLUSION: The differences in the air-bone gap at low and high frequency in pure-tone audiometry, static compliance, and acoustic reflex in impedance audiometry are useful for distinguishing OS from ISD. However, combinations of several examinations including computed tomography imaging are needed for the differential diagnosis of other ear diseases and specific preoperative planning.

Microscopic vs Endoscopic Ear Surgery for Congenital Ossicular Anomaly.

OBJECTIVE: To compare surgical outcomes of transcanal endoscopic ear surgery (TEES) for congenital ossicular anomalies with those of conventional microscopic surgery. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral academic center. SUBJECTS AND METHODS: From March 2012 to November 2018, 42 consecutive ears in 40 patients with congenital ossicular anomaly who underwent ossiculoplasty or stapes surgery using either ear endoscopes (TEES group) or an operating microscope (microscopic group) were included. Postoperative audiometric results, operation time, switch of approach, and complications were compared between the 2 groups. RESULTS: Twenty-four ears (66.1%) were in the microscopic group and 18 ears (33.9%) were in the TEES group. The mean (SD) preoperative air-bone gap was 31.8 (10.0) dB in the microscopic group and 35.2 (11.1) dB in the TEES group. The mean (SD) postoperative air-bone gap was 7.4 (6.5) dB in the microscopic group and 5.6 (5.0) dB in the TEES group. The differences in the preoperative and postoperative air-bone gaps between the 2 groups were not statistically significant (P = .316 and P = .412, respectively). Average operation time in the TEES group was 24.6 minutes shorter than that in the microscopic group, which was statistically significant (P = .019). None of patients in the TEES group did require a switch of approach. There was no significant difference in complication incidence between the 2 groups. CONCLUSIONS: TEES for congenital ossicular anomaly has comparable audiometric results and complication rates to conventional microscopic surgery. TEES appears to have the advantages of shorter operation times.

Ossicular chain reconstruction: endoscopic or microscopic?

OBJECTIVE: To compare the results of endoscopic and microscopic ossicular chain reconstruction surgery. METHODS: Patients undergoing ossicular chain reconstruction surgery via an endoscopic (n = 31) or microscopic (n = 34) technique were analysed for age, gender, Middle Ear Risk Index, ossicular chain defect, incision type, ossicular chain reconstruction surgery material, mean air conduction threshold, air-bone gap, air-bone gap gain, word recognition score, mean operation duration and mean post-operative follow up. RESULTS: Post-operative air conduction, air-bone gap and word recognition score improved significantly in both groups (within-subject p < 0.001 for air conduction and air-bone gap, and 0.026 for word recognition score); differences between groups were not significant (between-subject p = 0.192 for air conduction, 0.102 for air-bone gap, and 0.709 for word recognition score). Other parameters were similar between groups, except for incision type. However, endoscopic ossicular chain reconstruction surgery was associated with a significantly shorter operation duration (p < 0.001). CONCLUSION: Endoscopic ossicular chain reconstruction surgery can achieve comparable surgical and audiological outcomes to those of microscopic ossicular chain reconstruction surgery in a shorter time.

Cautionary Findings for the Presence of Facial Canal Dehiscence During Cholesteatoma Surgery.

Our aim was to investigate the relationship between facial canal dehiscence (FCD) and surgical findings and procedures in patients with cholesteatoma. A total of 186 patients (118 males, 39.2 ± 15 years) who underwent surgery for advanced cholesteatoma between 2013 and 2018 were included in the study. The relationship between FCD and surgical findings was investigated via the surgical registries. The prevalence of FCD was 36.6% (68/186). The prevalence of FCD was 44%, and 13.2% for the patients who underwent canal wall down mastoidectomy (62/141) and canal wall up mastoidectomy (6/45), respectively (P < .001). Facial canal dehiscence was detected in 73.9% of the 23 patients who had a lateral semicircular canal (LSCC) defect (P < .001), in 61.9% of 21 patients who had a tegmen tympani defect, and in 58.1% of the 31 patients who had erosion on the posterior wall of the external auditory canal (EAC; P < .05). The prevalence of FCD was 3.1% in patients with isolated incus erosion, 59.1% in patients with erosion of malleus and incus, 60.7% in patients with erosion of stapes suprastructure and incus, and 43.2% in patients with whole ossicular chain deformation (P < .001). The defects on LSCC, EAC, tegmen tympani, and malleus and incus might be cautionary findings for the presence of FCD during cholesteatoma surgery.

[Congenital middle ear malformation: clinical analysis and discussion of classification].

Objective: To analyze the clinical characteristics and appropriate surgical procedures, and discuss the classification of congenital middle ear malformation. Methods: All cases were from the Center of Otorhinolaryngology, the Sixth Medical Center of Department of PLA General Hospital. All of these cases, including 26 male patients (ears) , 10 female patients (11 ears) , aged from 7 to 57 years old, had normal external auditory canal, tympanic membrane, conductive hearing loss, type A tympanogram and negative Gelle's test. Tympanoplasty was performed in all cases. The deformity was classified to three types,i.e., Type I (stapes foot plate mobility): Ⅰa, ossicular chain deformity with normal stapes suprastructure; Ⅰb, ossicular chain deformity with abnormal stapes suprastructure; Type Ⅱ (stapes foot plate fixation): Ⅱ a,normal ossicular chain, Ⅱ b, ossicular chain malformation; and Type Ⅲ: vestibular window osseous atresia or undeveloped, or with round window atresia. The malformation of type Ⅱ and Ⅲ may be accompanied with abnormal facial nerve. In addition, the papers on middle ear malformation published from 1982 to 2017 were analyzed retrospectively. The clinical data of 451 ears malformation were summarized. Results: According to the revisional classification criteria in 37 ear samples from our hospital, 20 ears were type I. 6 type Ⅰa cases were used PORP (partial ossicular replacement prosthesis) to reconstruct the ossicular chain; 14 type Ⅰb cases were used TORP (total ossicular replacement prosthesis) to reconstruct the ossicular chain. For the 5 ears of type Ⅱ, 2 of which were type Ⅱ a and 3 were type Ⅱ b. 4 ear samples of type Ⅱ were implanted with Piston ossicular prosthesis, 1 was implanted with TORP in which the ossificated foot plate was removed with periosteum preserved. 12 ear samples were type Ⅲ, with vestibular window osseous atresia, facial nerve malformation, and stapes suprastructure malformation. The pistons ossicular prosthesis were implanted in vestibular window in 3 ears with facial nerve covering vestibular window partially. The surgery had to be given up in 5 ears, and TORP was implanted in 4 ears at the opening with preserved periosteum at the beginning of the tympanic scala because of facial nerve covering vestibular window totally. 30 ears with complete follow-up data had no sensorineural hearing loss and the average air-bone conduction decreased 23.3±10.7 dB (P<0.05).There were 234 ears of type Ⅰ in 451 ears of congenital middle ear malformation reported in the literature. 113 of which were type Ⅰa, the basic surgery was ossicular chain shaking and artificial or autogenous PORP implantation. Type Ⅰb was 121 ears, with autogenous or artificial TORP and PORP. Type Ⅱ was125 ears, including type Ⅱa 22 ears, Ⅱb 60 ears, and no subclassification for 43 ears. The surgery of type Ⅱ was the same as otosclerosis. The vestibular window atresia of type Ⅲ was 92 ears, the surgery of 17 ears had to be abandoned, the other ears underwent vestibular window, promontory or semicircular canal opening to reconstruct hearing with Piston, autogenous or artificial TORP. Conclusion: Referring to the classification of congenital middle ear malformation combining with appropriate surgical materials and methods, otologists can better understand and choose appropriate surgical method to the middle ear malformation.

Conductive hearing loss with a "dry middle ear cleft"-A comprehensive pictorial review with CT.

Conductive hearing loss (CHL) commonly results from middle ear fluid and inflammation (otitis media). Less commonly in patients with CHL, the middle ear cleft is well aerated or 'dry' with absence of soft tissue or fluid clinically and on imaging. There are numerous causes for this but they can be clinically challenging to diagnose. This pictorial review aims to illustrate and discuss the CT features of both common and less common causes of CHL in patients with a "dry middle ear cavity".

Reporting and Description for Congenital Middle Ear Malformations to Facilitate Surgical Management.

INTRODUCTION: The aim of this work was to report and describe the different types of congenital middle ear malformations in order to guide surgical treatment approaches and improve outcomes for affected patients. METHODS: The authors reviewed patients with congenital middle ear malformations who received surgical treatment between September 2010 and March 2017. Patient characteristics, middle ear deformities, and surgical procedures were documented. RESULTS: In this retrospective study, 35 patients were reviewed. A description of middle ear malformation was proposed that considers ear embryogenesis and focuses on stapes deformity, with the main purpose of facilitating surgical approach selection to reconstruct the ossicular chain. Patients were classified into 3 categories: type I (19 cases), mobile stapes footplate, which included type Ia with normal stapes suprastructure and type Ib with abnormal stapes suprastructure; type II (4 cases), fixed stapes footplate, which included type IIa with normal ossicular chain and type IIb with abnormal ossicular chain; and type III (12 cases), oval window bony atresia or aplasia, with or without round window atresia. Types II and III could have concomitant aberrant facial nerve. Different surgical approaches are described. CONCLUSIONS: The authors describe the different types of congenital middle ear malformations. This category description considers ear embryogenesis and is focused on stapes deformity. It may provide better understanding of disease development and guide modern hearing reconstructive surgery.

Anatomical measurement of the ossicles in patients with congenital aural atresia and stenosis.

OBJECTIVES: Our aims were to measure and compare anatomical parameters of the ossicles in normal, congenital aural stenosis (CAS), and congenital aural atresia (CAA) ears. METHODS: This retrospective study was performed using three-dimensional reconstructed images derived from computed tomography scans of 20 normal subjects, 20 CAS patients, and 20 CAA patients. RESULTS: The lengths of the malleus handle and long process of the incus were greater in normal ears than in CAS and CAA ears (all P < 0.05). The angles of the incudostapedial joint and between the short and long processes of the incus were smaller in normal ears than in CAS and CAA ears (all P < 0.05). There were no significant differences in the positions of the malleus head and incudomalleolar joint, the size of the malleus head, the length of the short process of the incus, or the angle of the incudomalleolar joint (P > 0.05). CONCLUSIONS: Anatomical parameters of the lower part, but not of the upper part, of the ossicular chain in CAS and CAA ears differed from those in normal ears. Different branchial arch origins of the upper and lower parts of the ossicular chain may explain these findings. Dysplasia of the second arch, which develops into the lower part of the ossicular chain, may contribute to ossicular malformation in CAA and CAS. Accurate radiographic measurement of malformed ossicles may be useful for reconstructive surgery of CAA and CAS using the patient's native ossicular chain and for choosing an appropriate place for active middle ear implants.

Autologous incus versus titanium partial ossicular replacement prosthesis in reconstruction of Austin type A ossicular defects: a prospective randomised clinical trial.

OBJECTIVE: This study aimed to compare the functional and anatomical outcomes of ossiculoplasty using an autograft incus or a titanium partial ossicular replacement prosthesis for reconstructing Austin type A ossicular defects. METHODS: Patients with Austin A ossicular defects were randomly divided into two groups: one group underwent ossiculoplasty with an autologous incus (the autologous incus group) and the other underwent ossiculoplasty with a titanium partial ossicular replacement prosthesis (the titanium prosthesis group). Otoscopic examination and audiological assessment was done pre-operatively and at 3, 6 and 12 months post-operatively. RESULTS: A post-operative average air-bone gap closure of less than 20 dB was seen in 13 patients (65 per cent) in the autologous incus group and 7 (35 per cent) in the titanium prosthesis group. There were fewer post-operative complications in the autologous incus group (20 per cent) than in the titanium prosthesis group (45 per cent). CONCLUSION: Hearing outcomes and graft take up after ossiculoplasty were significantly better when an autologous incus rather than a titanium partial ossicular replacement prosthesis was used to reconstruct Austin type A ossicular defects. The major disadvantages of the titanium prosthesis were unpredictable results and more post-operative complications.

Congenital cholesteatoma together with ossicular chain anomaly.

Cholesteatomas that occur under an intact tympanic membrane in the absence of prior surgical procedures or perforation are defined as congenital cholesteatomas. These entities are rarely seen, because they do not cause any major symptoms unless they touch the ossicular chain. Likewise, isolated congenital ossicular anomalies that occur independently of external ear anomalies and craniofacial dysplasia are also rarely seen. Here, we report a patient who presented with congenital cholesteatoma associated with anomalies of the ossicular chain and discuss its pathogenesis.