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Objective: Little is known about the hearing characteristics in patients with congenital round window atresia (CRWA). This study aimed to investigate hearing characteristics in patients with CRWA by comparing them with two relatively common congenital middle ear anomalies: congenital stapedial fixation (CSF) and congenital ossicular discontinuity (COD)., Methods: Literature searches yielded five patients with surgically confirmed CRWA (seven ears), who were included in the CRWA group, along with one of our patients. Air and bone conduction thresholds; air-bone gap (ABG); and presence and depth of the Carhart notch were analyzed. These audiometric variables in the CRWA group were compared with those in the CSF (n = 15) and COD (n = 22) groups, comprising patients identified from our institution's medical database., Results: Average bone and air conduction thresholds in the CRWA group were 16.4 (standard deviation [SD]: 2.9; 95 % confidence interval [CI]: 14.6-18.3) and 44.6 (SD: 3.5; 95 % CI: 42.6-47.3) dB hearing level (HL). Bone conduction thresholds at high frequencies (≥2 kHz) were higher than those at low frequencies (<2 kHz), while air conduction thresholds at high frequencies were lower than those at low frequencies: ABGs at high frequencies were significantly smaller than those at low frequencies (2 kHz vs. 0.5 kHz, p = 0.027; 2 kHz vs. 1 kHz, p = 0.041; 4 kHz vs. 0.5 kHz, p = 0.042; 4 kHz vs. 1 kHz, p = 0.027). There were no between-group differences in incidence and depth of the Carhart notch., Conclusion: CRWA could manifest as a distinct audiometric pattern with poorer bone conduction and better air conduction at ≥2 kHz, resulting in significantly smaller ABGs at higher frequencies than that at lower frequencies. Our findings indicated that this pattern differed from that of CSF and COD. The unique beer bottle-shaped audiogram associated with CRWA might facilitate its early diagnosis in patients with congenital conductive hearing loss., Competing Interests: Declaration of competing interest The authors declare that they have no competing interests., (Copyright © 2024 Elsevier B.V. All rights reserved.)

Objective: To explore the surgical efficacy of conductive deafness caused by otosclerosis and ossicular malformation with 980 nm fiber laser stapedial floor fenestration. Methods: Data of 58 patients (ears) who were diagnosed with conductive deafness caused by otosclerosis (49 ears) and ossicular malformation (9 ears) treated by 980 nm Diode laser small-fenestra stapedotomy were retrospectively analyzed. Air conduction (AC) thresholds, bone conduction (BC) thresholds, and air-bone gap (ABG) at 0.5, 1, 2, 4 kHz pure tone frequencies were compared before and 3 months after surgery, and the advantages and precautions of 980 nm fiber laser were summarized. Paired t -test (SPSS 26.0 software) was use to analyze the listening data. Results: Fiber optic laser stapes fenestration and artificial stapes implantation were successfully completed in all 57 cases (ears), the hearing of another one patient (ear) with floating malformation of detachment of stapedial floor was lower than that before surgery. Preoperative at 0.5, 1, 2, 4 kHz frequencies of AC thresholds, BC thresholds, and ABG were (65.4±9.7) dB, (27.2±8.9) dB, and (38.2±9.8) dB respectively. Postoperative 3 months at the same frequency of AC thresholds, BC thresholds, and ABG were (42.1±11.3) dB, (26.9±6.6) dB, and (15.2±9.1) dB. Preoperative and postoperative of AC threshold and ABG were statistically significant at 0.5, 1, 2, 4 kHz ( t value was 13.270 and13.948, both P <0.01), and yet the BC threshold was not statistically significant before and after surgery at the same frequency ( t =0.418, P >0.05). Conclusions: 980 nm fiber laser stapes floor fenestration is an effective treatment for conductive deafness caused by otosclerosis and ossicular malformation.

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., Competing Interests: Declaration of Competing Interest The authors have no funding, financial relationships, or conflicts of interest to disclose., (Copyright © 2023. Published by Elsevier B.V.)

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., (© 2023 American Academy of Otolaryngology-Head and Neck Surgery Foundation.)

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.

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., Competing Interests: The authors disclose no conflicts of interest., (Copyright © 2022, Otology & Neurotology, Inc.)

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.

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.

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., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2020. Published by Elsevier B.V.)

Background: Spondyloepiphyseal dysplasia congenita (SEDC) is an autosomal dominant chondrodysplasia characterized by disproportionate short stature, abnormal epiphyses, and flattened vertebral bodies. COL2A1 has been confirmed as the pathogenic gene. Hearing loss represents an infrequent manifestation for 25-30% of patients with SEDC. The characteristics of the hearing impairment were rarely documented., Methods: Audiological, ophthalmic, imaging examinations were conducted on the family members. The whole exome sequencing (WES) was performed to detect the candidate gene, and the Sanger sequencing was used to confirm the causative variation., Results: COL2A1 c.1510G>A (p.G504S), a hot spot variation, was identified as the disease-causing mutation of the Chinese Li nationality family with SEDC. This variation was co-segregated with the SEDC phenotype in the family and was absent in the 1000 Genomes Project, ESP and ExAC. Clinically, several manifestations were first demonstrated in SEDC patients caused by p.G504S, including sensorineural hearing loss, auditory ossicles deformity, retinal detachment, sacrum cracked and elbow and wrist joints deformity. Other classical SEDC manifestations such as bones and joints pain, midfacial dysplasia, disproportionate short stature, spinal deformity, thoracocyllosis, coxa arthropathy, myopia and waddling gait were also showed in the family patients., Conclusion: We first identified the mutation p.G504S in COL2A1 gene as the pathogenesis in a Chinese Li nationality family and reported the correlation between p.G504S and atypical clinical phenotypes including sensorineural hearing loss, auditory ossicles deformity, retinal detachment, sacrum cracked and elbow and wrist joints deformity. Our findings would extend the phenotypic spectrum of SEDC and deepen clinicians' understanding of genotype-phenotype correlation of the disease.

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.

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.

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.

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.

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., Competing Interests: Declaration of Competing Interest The authors declare no sources of financial support or conflicts of interest., (Copyright © 2020. Published by Elsevier B.V.)

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.

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.

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 (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"., (Crown Copyright © 2018. Published by Elsevier B.V. All rights reserved.)

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.

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., (Copyright © 2017. Published by Elsevier B.V.)

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.

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.

Objectives/hypothesis: To review surgical findings and hearing outcomes of incus footplate assembly (IFA) for the patients with conductive hearing loss due to missing stapes superstructure with a mobile stapes footplate., Study Design: Retrospective case review and survey., Methods: Pre- and postoperative audiometric data and intraoperative findings were reviewed. Postoperative air-bone gap (ABG) and ABG closure (postoperative air-conduction threshold-preoperative bone-conduction threshold) were analyzed., Results: The causes of missing stapes superstructure and conductive hearing loss were congenital ossicular anomaly (n = 5), chronic otitis media (n = 2), and congenital cholesteatoma (n = 1). The prosthesis was designed to fit between the medial side of the incus and stapes footplate and had a mean length of 3.6 ± 0.5 mm. The mean pre- and postoperative ABG were 38.3 ± 4.8 and 13.3 ± 10.0 dB, respectively. The postoperative ABG at frequencies of 0.25, 0.5, 1.0, 2.0, 3.0, and 4.0 kHz were 20.0 ± 15.4, 16.9 ± 11.9, 16.3 ± 10.3, 10.6 ± 7.3, 12.9 ± 14.0, and 23.1 ± 16.2 dB, respectively. The mean ABG closure was 9.5 dB (range, -1.3∼35.8 dB). Seven cases obtained the best results (mean ABG closure ≤10 dB). In the remaining patient, the mean ABG closure was 9.5 dB until 6 months after surgery, but was 35.8 dB 1 year after surgery., Conclusions: IFA seems to be a reasonable surgical option in patients with missing the stapes superstructure, but with a mobile footplate in which the long process of incus is preserved., Level of Evidence: 4 Laryngoscope, 126:2569-2573, 2016., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: To determine the optimal surgical approach for cochlear implantation (CI) preoperatively based on the spatial relation of a displaced facial nerve (FN) and middle ear structures and to analyze clinical outcomes of CHARGE syndrome., Methods: Facial nerve displacement and associated deviation of inner ear structures were analyzed in 13 patients (17 ears) with CHARGE syndrome who underwent CI. Surgical accessibility through the facial recess was assessed based on anatomical landmarks. Postoperative speech performance and associated clinical characteristics were analyzed., Results: The most consistently identified ear anomalies were semicircular canal aplasia (100%), ossicular anomaly (100%), and vestibular hypoplasia (88%). Facial nerve displacement was found in 77% of cases (anteroinferior: 47%, anterior: 24%, inferior: 6%). The width of available surgical space around facial recess was significantly greater in cases of facial recess approach (2.85 ± 0.9 mm) than those of alternative approach (0.12 ± 0.29 mm, P = .02). Postoperatively, 53% achieved better than category 4 on the categories of auditory perception (CAP) scale. The CAP category was significantly correlated with internal auditory canal diameter (P = .025) and did not differ according to the applied surgical approach., Conclusion: Preoperative determination of surgical accessibility through facial recess would be useful for safe surgical approach, and successful hearing rehabilitation was achievable by applying appropriate surgical approaches., (© The Author(s) 2016.)

Objectives: 1) To describe the technique for transcanal endoscopic management of congenital ossicular chain fixation. 2) To highlight the utility and outcomes of the endoscopic approach for management of congenital ossicular fixation., Study Design: Retrospective patient series., Setting: Academic tertiary pediatric hospital., Patients: Pediatric patients (age 6-12) undergoing transcanal endoscopic management of congenital ossicular fixation from May 2014 to December 2014., Interventions: A transcanal endoscopic approach was used in eight procedures. Ossicular chain pathology was managed with either mobilization, ossiculoplasty with a stapes prosthesis, or incus interposition., Main Outcome Measures: Pure-tone averages, speech reception thresholds, and speech discrimination scores were recorded pre- and postoperatively for each subject. Preoperative computed tomography evaluations were compared to intraoperative findings for each subject., Results: An improvement in the pure-tone average, as well as air-bone gap, was noted after six of eight procedures. No patients experienced a complication or a reduction in their bone conduction hearing. The chorda tympani nerve was preserved in all eight patients. Conversion to open approach was not necessary for any of the eight procedures performed., Conclusion: The transcanal endoscopic approach was successful in improving hearing in pediatric patients with congenital ossicular fixation that involves any of the three ossicles. An endoscopic transcanal approach provides an alternative method to manage congenital ossicular pathology with the advantage of providing excellent visualization and the avoidance of a postauricular incision.

Objective: We analyzed audiologic and surgical findings in patients with conductive hearing loss (CHL) with an intact tympanic membrane (TM) that was of a non-inflammatory origin., Methods: We reviewed data from patients who underwent exploratory tympanotomy for CHL with intact TM from January 1995 to November 2012. Patients with diseases of non-inflammatory origin were enrolled (69 patients; 79 ears). Patients were categorized into two groups: non-trauma (50 ears) and trauma (29 ears). Demographic data, intraoperative findings, and audiologic results were obtained and analyzed., Results: Overall, the second decade was the most common age of diagnosis in both the non-trauma and trauma groups. Operative findings showed that ossicular dislocation was more prevalent than ossicular fixation; all trauma group subjects had ossicular dislocation. Short columellization or partial ossicular replacement was the most frequently adopted surgical procedures in both groups. Overall, audiologically, air-conduction thresholds (ACs) and air-bone gaps were significantly improved over the short- and long-term period in both groups. However, the non-trauma group had significantly higher preoperative ACs than the trauma group, especially at low frequencies., Conclusion: This study provides clinicians with useful information regarding the clinical characteristics of CHL with intact TM of non-inflammatory origin., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Objectives/hypothesis: To prospectively evaluate surgical findings and hearing results in children undergoing surgery for congenital malformations of the ossicular chain with a mobile stapes footplate (Teunissen and Cremers class III malformations)., Study Design: A nonrandomized, nonblinded, prospective case series., Methods: Fifteen consecutive pediatric patients who underwent 17 surgical procedures for congenital ossicular malformations with a mobile stapes at a tertiary referral center were included. In 16 cases, postoperative pure-tone audiometry was available. The surgical technique for repair of the ossicular chain was dictated by the surgical findings at the time of surgery. The majority of the cases underwent ossiculoplasty. A Teflon piston, partial ossicular replacement prosthesis, or total ossicular replacement prosthesis was used in these cases. Associated surgical techniques included malleus relocation, Silastic banding, drilling out of a bony bridge, and a combination of these techniques. Hearing loss was evaluated using preoperative and postoperative four-frequency (0.5, 1, 2, and 4 kHz) audiometry. Air-conduction thresholds, bone-conduction thresholds, and air-bone gaps were measured. Postoperative audiometry was performed at 3, 6, 9, 12, 18, and 24 months after surgery and at a yearly interval thereafter., Results: Overall, a postoperative air-bone gap closure to 10 dB or less was achieved in 63% of the included cases. A postoperative air-bone gap closure to 20 dB or less was achieved in 75%. Postoperative sensorineural hearing loss did not occur in any of the cases., Conclusions: Ossicular reconstruction is a feasible treatment option in children with congenital malformations of the ossicular chain with a mobile stapes footplate., Level of Evidence: 4. Laryngoscope, 126:682-688, 2016., (© 2015 The American Laryngological, Rhinological and Otological Society, Inc.)

Ear abnormalities in oculo-auricular-vertebral spectrum commonly present with varying degrees of external and middle ear atresias, usually in the expected locations of the temporal bone and associated soft tissues, without ectopia of the external auditory canal. We present the unique imaging of a 4-year-old girl with right hemifacial microsomia and ectopic location of an atretic external auditory canal, terminating in a hypoplastic temporomandibular joint containing bony structures with the appearance of auditory ossicles. This finding suggests an early embryological dysfunction involving Meckel's cartilage of the first branchial arch.

Summary: A subset of patients presents with unilateral conductive hearing loss, a normal pinna or grade I microtia, and mild external auditory canal stenosis. The physical findings of microtia and a small external canal are commonly absent or subtle in this group of patients, who are being commonly referred for imaging to evaluate isolated conductive hearing loss. We present a case series of patients with unilateral conductive hearing loss and characteristic ossicular abnormalities, commonly anterior fixation of the malleus. All patients had a significantly increased distance from the cochlear promontory to the handle of the malleus and an abnormal incudostapedial angle, indicative of an abnormal ossicular position and/or morphology. Successful surgical reconstruction of the ossicular chain was attempted and accomplished in 3 patients., (© 2015 by American Journal of Neuroradiology.)

The aim of this study was to describe the audiometric results following surgery in a consecutive series of pediatric patients with a congenital middle ear disorder. Retrospective chart review was performed for 29 consecutive children who underwent 33 middle ear surgeries for congenital ossicular chain anomaly between 1990 and 2012. Anomalies were classified into four groups according to the Teunissen and Cremers classification. Audiological parameters using four frequency averages (0.5, 1, 2 and 4 kHz) were assessed pre- and postoperatively. Clinical and audiometric follow-up times were, respectively, 49 ± 8 and 35 ± 5 months (mean ± SEM). Fifty-eight percent of all patients achieved an air-bone gap (ABG) ≤20 dB, 62.5% in class I, 50% in class II and 57.9% in class III. The improvement of the mean ABG was 13.6 dB, 19.2 dB for class I, 0.2 dB in class II and 15.4 dB in class III. Overall mean pure-tone averages improved 14.8 dB with 13.9 dB for class I; there was no improvement for class II and 20.2 dB for class III. The sensorineural hearing loss rate was 9%. This pediatric series showed that hearing results depend on type of anomaly. Class I and class III showed better hearing improvement than class II., (© 2015 S. Karger AG, Basel.)

Choristoma of the middle ear is a rare condition characterized by the presence of normal salivary gland tissue in the middle ear space. Salivary gland choristomas are benign lesions that are frequently associated with ossicular chain and facial nerve anomalies. Total surgical excision is indicated when there is no risk of damaging the facial nerve. We describe a new case of salivary gland choristoma of the middle ear, and we discuss the etiology, histologic features, and management of such lesions. Our patient was a 22-year-old woman in whom we surgically removed a whitish retrotympanic mass. Intraoperatively, we also detected an ossicular chain malformation. Histologic examination of the choristoma revealed the presence of salivary gland tissue. Furthermore, the lesion contained an extensive and previously undescribed component: a well-defined pseudostratified respiratory-type epithelium, similar to that of a normal eustachian tube. Ten months after removal of the choristoma, we surgically repaired the ossicular chain anomalies. No recurrence was noted on follow-up.

Objective: The objective was to elucidate clinical effectiveness of submillimeter multislice 2- and 3-dimensional computed tomography (CT) for diagnosis of congenital middle ear anomaly., Materials and Methods: Preoperative CT and operative findings were retrospectively analyzed in 36 ears of 33 patients. The distance between the malleal handle (MH) and the incus long process (ILP) was measured in 28 normal ears., Results: Mean ± SD MH-ILP distance in normal ears was 1.8 ± 0.3 mm. In patients with middle ear anomaly, overall specificity, accuracy, and positive and negative predictive values were 99.7%, 98.7%, 91.7%, and 99.1%, respectively. Overall sensitivity was 82.1%, with relatively low sensitivities for detecting fused incudomalleolar joint, lenticular process defect, stapedial footplate defect, and oval window atresia and high sensitivities (80%-100%) for stapedial crural anomaly and certain fibrous connections., Conclusions: Submillimeter CT improved detectability of anomalies of the stapedial superstructure; however, it is still difficult to identify abnormalities of the footplate.

The majority of the congenital anomalies of middle ear are solitary and a non-hereditary. We report cases of identical twins with congenital incudo-stapedial disconnection. Case 1 was an 8-year-old girl. Hearing impairment was identified at the age of three. She was referred to our university hospital in April 2005. Pure-tone audiogram showed conductive hearing impairments. Computed tomography (CT) revealed the incudo-stapedial disconnections in both ears. The exploratory tympanotomies on the right and left ears were performed in May and July 2005, respectively. The surgical findings showed absence of the long process and presence of the lenticular process of the incus in both ears. After the reconstructions of ossicular chain, the hearing of both ears improved. Case 2 was an 11-year-old girl. The hearing impairment of the right ear was identified in May 2008. She was referred to our university hospital three months later. Pure-tone audiogram showed the conductive hearing impairment in the right ear. CT revealed the incudo-stapedial disconnection in the right ear. The surgery showed the same findings as those of case 1. Anomalies of both cases suggest that the lenticular process of the incus and the stapes originate from a common primordium., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Objective: The aim of this article was to assess the prevalence and influence of anomalous facial nerves in patients undergoing surgery for congenital stapes anomalies., Patients: The medical records of all patients who underwent surgery for congenital stapes fixation between January 1999 and December 2012 were retrospectively reviewed. An abnormal facial nerve was found in 7 ears in 5 patients (from a total of 62 ears)., Main Outcome Measures: The patients' temporal bone computed tomography results, operation records, video clips, and preoperative and postoperative hearing tests were reviewed., Results: Oval window atresia was found in all 7 ears, and the facial nerves in all 7 ears ran inferior medially over the atretic oval window niche. The atretic plate was fenestrated into the vestibule in the superior margin of the facial nerve. A piston wire was anchored to the long process of the incus in 3 ears, to the handle of the malleus in 3 ears, and attached to the tympanic membrane in 1 ear. The postoperative ABG at long-term follow-up was 13.1 dB (SD, 4.6 dB; range, 8.8-18.3 dB). No patient had postoperative facial weakness., Conclusion: An anomalous facial nerve was found in 11.2% of ears in patients undergoing surgery for congenital stapes fixation. Despite the anomalous course of facial nerves in these patients, vestibular fenestration was successful without facial nerve damage. The audiometric outcome at long-term follow-up was serviceable hearing for all cases.

CHARGE syndrome is a rare, polymalformative disease, representing one of the major causes of associated blindness and deafness. Bilateral, severe-profound, sensorineural hearing loss is common in CHARGE children. Aim of this study is to present our results in children with "CHARGE syndrome" submitted to cochlear implantation (CI). The frequency of anatomic anomalies, possible variations in the surgical technique of CI, and the audiological/rehabilitative benefits attained in our patients are reported. we submitted 5 children affected by CHARGE syndrome with profound, bilateral, sensorineural hearing loss to CI. Otoacoustic emissions, auditory brainstem response, acoustic impedance testing, cranial computed tomography and magnetic resonance were carried out preoperatively in all children. CI was performed using the mastoidotomy-posterior tympanotomy approach in two cases, and the suprameatal approach in three children. Infant toddler-meaningful auditory integration scale was used to evaluate kid's audiological performance before and after CI. Intra-operatory findings and postsurgical complications were evaluated. Among our patients, intraoperative anatomical malformations were cochlear hypoplasia (100 %), ossicles malformations (100 %), semicircular canals aplasia (100 %), oval window atresia (60 %), round window atresia (40 %), widening of the aqueduct of the vestibule (20 %), and aberrant course of the facial nerve (20 %). No intra- or postoperative complication was recorded in relation to implant positioning. After a follow-up ranging from 1 to 4.5 years, only 2/5 patients used oral language as the sole mean of communication, 1 started utilizing oral language as the main mean of communication, while 2 patients did not develop any linguistic ability. In conclusion, CI in patients with CHARGE association is feasible and, despite results variability, it should be carried out in CHARGE children with severe hearing loss as soon as possible. Although the selection of a specific surgical technique does not seem to influence the audiological outcome, the suprameatal access is valuable when important surgical landmarks (i.e. lateral semicircular canal and incus) are absent.

Generally reporting, among the various forms of conductive hearing loss, auditory ossicular malformation clinically treated by surgery had good hearing improvement. We conducted a retrospective review of 40 patients (44 ears) with auditory ossicular malformations who were treated in our hospitals between April 2004 and March 2011. We analyzed the following preoperative features, surgical methods, and results of surgery. An otomicroscopic examination, auditory ossicules reflection, tympanometory, and temporal bone high-resolution computed tomography were undertaken in all patients. We also investigated whether these preoperative examinations would enable surgeons to make a preoperative diagnosis. There were 13 males (14 ears) and 27 females (30 ears), with an average age of 19.0 years. Classification of the pathologic condition based on surgical findings showed separation of the incus-stapes joint in 24 ears, fixation of the malleus or incus in 6 ears, fixation of the stapes footplate in 7 ears, and multifocal ossicular malformations in 7 ears. Ossicular reconstruction was performed by the modified type III method in 27 ears (including IIIc in 21 ears, IIIi-M in 1 ears, IIIi-I in 5 ears) and by the modified type IV method in 7 ears (including IVc in 5 ears, and IVi-I in 2 ears), stapes surgery in 11 ears (include total stapedectomy in 9 ears and partial stapedectomy in 2 ears) and exploratory tympanotomy in 1 ear. Postoperative hearing evaluations based on the criteria classified by the Japan Otology Society in 2010 were obtained for all cases. The procedure was deemed successful when the postoperative hearing level met at least one of these three bench marks; (1) Air-bone gap less than 15dB, (2) Recovered hearing more than 15dB, and (3) Improved or preserved hearing less than 30dB. Hearing was evaluated at 1 year after surgery. The success rates of hearing improvement was 92.3%. The success rates of postoperative hearing improvement were satisfactory. Surgeons should treat auditory ossicular malformations actively.

Understanding of the embryologic origin of the stapes remains controversial. Theories diverge upon whether the entirety of the stapes arises from a single source versus the footplate and suprastructure arising from distinct sources. A 12-year-old boy with left-sided conductive hearing loss had computed tomography of the temporal bone, revealing an inferiorly displaced left stapes, and a nonspecific density in the left Prussak's space. Exploratory tympanotomy revealed the crura of the stapes to be attached to the promontory. The stapes footplate was located in the oval window and was mobile., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objective: We explore the deformity degrees of external ear, mastoid process, tympanic cavity and auditory ossicle by the HRCT features of bilateral and unilateral patients of congenital abnormality of external and middle ear for preoperative analysis and selection for surgical approaches., Method: Twenty-nine patients were enrolled in our study, and HRCT were performed on all patients. There were 23 unilateral ears deformity (13 right and 10 left), 6 patients with bilateral ears deformity in the study group. Nineteen patients (19 ears) were treated with operations., Result: The deformity degree of auricle was correlated with that of external auditory meatus, and mastoid process develops bad aeration extent with bad tympanic cavity development in 35 ears in our research. We got data of shortest distance from tympanic cape to atresia board in HRCT, (0.59 +/- 0.13)cm in comparison group, (0.45 +/- 0.19)cm in unilateral deformity group, (0.32 +/- 0.12)cm in bilateral deformity group, and there were significant differences compared the two deformity groups with the comparison group. There were 1 ear with normal auditory ossicle (2.86%), 5 ears with fixation of stapes foot board (14.29%), 3 ears with no auditory ossicle (8.57%); 26 ears with malleus and incus abnormality (74.29%). Abnormality of malleus was always concomitant with that of incus. Degeneration of malleus, the joint amalgamation of malleus and incus were discovered mostly in this research., Conclusion: The deformity degree of auricle is correlated with that of external auditory meatus, and mastoid process develops bad aeration extent with bad tympanic cavity development in 35 ears in our research. The shortest distance from tympanic cape to atresia board in HRCT in unilateral ears deformity group are shorten 0.15 cm in that of comparison group. And that of bilateral ears deformity group are shorten 0.25 cm in that of comparison group. The main abnormality of auditory ossicle in our research present in both malleus and incus, and degeneration of malleus and the joint amalgamation of malleus and incus are main types.

Prolyl 3-hydroxylase1 (P3H1) is a collagen modifying enzyme which hydroxylates certain prolines in the Xaa position of conventional GlyXaaYaa triple helical sequence. Recent investigations have revealed that mutations in the LEPRE1 (gene encoding for P3H1) cause severe osteogenesis imperfecta (OI) in humans. Similarly LEPRE1 knockout mice display an OI-like phenotype. Significant hearing loss is a common problem for people with osteogenesis imperfecta. Here we report that hearing of the P3H1 null mice is substantially affected. Auditory brainstem responses (ABRs) of the P3H1 null mice show an average increase of 20-30 dB in auditory thresholds. Three dimensional reconstructions of the mutant middle ear bones by Micro-scale X-ray computed tomography (Micro-CT) demonstrate abnormal morphology of the incudostapedial and incudomalleal joints. We establish the LEPRE1 knockout mouse as a valuable model system to investigate the mechanism of hearing loss in recessive OI., (Copyright © 2012 International Society of Matrix Biology. Published by Elsevier B.V. All rights reserved.)

Aim: To investigate the aetiology and clinical consequences of incudo-stapedial (IS) discontinuity when it is demonstrated on computed tomography (CT) within a fully aerated middle ear and mastoid., Methods and Materials: Patients with CT evidence of IS discontinuity within a fully aerated middle ear and mastoid were prospectively identified. Clinical history, otoscopic findings, audiometry, and CT data were evaluated. Predefined criteria were used to determine the likely aetiology of IS discontinuity, whether it was diagnosed prior to the CT study, and the clinical consequences in terms of degree of conductive hearing loss and requirement for surgical correction. The range of CT appearances was evaluated., Results: The IS discontinuity in 34/36 ears was felt to be due to incus erosion secondary to chronic otitis, on the basis of clinical history and otoscopic findings. The IS discontinuity was rarely evident prior to CT with long-process deficiency being identified in only 5/36 cases. The mean air bone gap was only 22.5 dB. The ossicular defect was surgically addressed in only four cases. The incus deficiency was confined to the lower-third on CT in 19/36 cases., Conclusion: When IS discontinuity is demonstrated within a fully aerated middle ear and mastoid, the most likely aetiology is of acquired incus erosion due to chronic otitis media. The IS discontinuity on CT is usually not evident otoscopically. It usually results in only mild conductive hearing loss and the ossicular discontinuity was rarely surgically addressed in the present series., (Copyright © 2012 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Objectives: To describe the audiometric results in a consecutive series of patients with congenital ossicular aplasia (Class 4a) or dysplasia of the oval and/or round window (Class 4b), which might include a possible anomalous course of the facial nerve., Study Design: Retrospective chart study., Setting: Tertiary referral center., Patients: A tertiary referral center study with a total of 14 patients with congenital minor ear anomalies as part of a consecutive series (n = 89) who underwent exploratory tympanotomies (15 ears)., Main Outcome Measures: Audiometric results., Results: In 8 of 15 ears, ossicular reconstruction was attempted. In the short term (1 mo), there was a serviceable hearing outcome (air-bone gap closure to within 25 dB) in 4 ears. However, the long-term results showed deterioration because of an increased air-bone gap in all but 1 ear. No facial nerve lesion was observed postoperatively., Conclusion: Congenital dysplasia or aplasia of the oval and/or round window is an uncommon congenital minor ear anomaly. Classical microsurgical opportunities are rare in this group of anomalies. Newer options for hearing rehabilitation, such as the osseointegrated passive bone conduction devices, have become viable alternatives for conventional air conduction hearing devices. In the near future, upcoming active bone conduction devices might become the most preferred surgical option. In cases in which the facial nerve is only partially overlying the oval window, a type of malleostapedotomy procedure might result in a serviceable postoperative hearing level.

Conclusion: In patients with cochleovestibular malformations, surgeons should always consider the possibility of an aberrant course or shape of the facial nerve (FN). In our series, the majority of cases could be successfully implanted without major complications through careful handling of the FN and ossicles., Objectives: To assess the frequency, type, and surgical implications of FN aberrations encountered during cochlear implantation (CI)., Methods: Medical records and radiologic findings of 972 patients who underwent CI from 1988 to 2009 were reviewed retrospectively. Radiologic and intraoperative findings demonstrating malformations of the FN, ossicles, and inner ear were evaluated. Intraoperative events and postoperative FN outcome were reviewed., Results: Seven patients (0.7%) had an aberrant course or shape of the FN during CI. All these patients were pediatric. Four showed abnormal anterior or inferior location of the vertical segment, two had a bifurcated horizontal or vertical segment, and one had an inferiorly located horizontal segment. All seven patients displayed combined malformations of the cochlea and had malformed or missing ossicles. CIs were successfully performed via the facial recess approach and cochleostomy. There was no postoperative FN paralysis except in one case with immediate iatrogenic FN paralysis that recovered completely 1 month after FN decompression.

Three patients with a middle ear malformation characterized by the absence of the oval window and complicated by facial nerve anomalies were treated by surgery. Hearing ability of all the patients was improved. A variety of facial nerve anomalies were observed. Fenestration of the vestibule above the facial nerve was performed in the case of 2 of the 3 patients. In the other patient, fenestration of the scala vestibuli below the facial nerve was performed. In the case of all the 3 patients, hearing ability improved after the surgery and remained stable for a long time. Surgery for this disease has been considered difficult because of a high risk of complications. Because all the 3 patients showed excellent long-term surgical results, we suggest that fenestration of the inner ear should be considered one of the main options for the management and treatment of an absent oval window associated with facial nerve anomalies., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Objectives: We describe the audiometric results following surgery in a consecutive series of patients with a congenital ossicular middle ear disorder that was associated with a mobile stapes footplate., Methods: We performed a retrospective analysis of patient charts from a tertiary referral center. A total of 23 patients (23 ears) underwent exploratory tympanotomy and ossicular reconstruction between 1986 and 2001. The main outcome measure was the audiometric results., Results: Overall, we observed a mean gain in air conduction pure tone average of 17 dB (from 47 dB to 30 dB), a sensorineural deterioration of 3 dB, and a mean postoperative air-bone gap of 19 dB (mean preoperative air-bone gap of 38 dB). The air-bone gap closure was 20 dB or less in 15 of the 23 cases (65%), in agreement with the few results reported in the literature. Moreover, the audiometric results remained stable. In the syndromic group, the mean gain in air conduction was only 13 dB, which was worse than that observed for the nonsyndromic ears., Conclusions: Surgery for congenital ossicular chain anomalies with a concomitant mobile stapes footplate provides positive audiometric outcomes. Most ears had some sensorineural impairment (10 to 20 dB), which influenced the final hearing level attained after surgery. Preoperative assessment is mandatory to search for syndromal diagnoses, which might be important for patient counseling and prognosis.

We retrospectively analyzed 26 ears of 21 subjects having auditory ossicular malformation and who had undergone auditory reconstruction between April 2004 and December 2010 at our clinic. We checked preoperative condition, pathological classification, surgical procedure, and hearing improvement. We could predict pathological conditions precisely from preoperative computed tomography (CT), including incudostapedial disconnection (9/12, 75%) and malleus and/or incus fixation (7/12, 58%), which tended to be present in external ear malformation, and stapes footplate fixation (0/12, 0%). We could not, however, predict complex malformation (0/8, 0%). Overall success was 90% (18/20) in the 20 ears observed for at least 1 year. In the 2 ears without improved hearing, the first had congenital cholesteatoma and no stapes superstructure, was treated with type IV tympanoplasty. The second had malleus, incus, and stapes fixation and discontinuity between the incus and stapes, and was treated with type III tympanoplasty and stapes mobilization. Preoperative diagnosis is difficult in mixed congenital auditory ossicular malformation, especially stapes footplate fixation, possibly requiring unexpected procedures, with a poor hearing outcome. Preoperative status must thus be evaluated precisely using hearing, tympanometry, acoustic reflex test, and CT. Temporal bone CT and external ear findings are useful in diagnosing middle-ear malformation. Subjects' informed consent should also be obtained due to the possible need for changing procedure based on findings during surgery.

Objectives: The management of developmental anomalies of the ossicular chain and otic capsule in stapes surgery tests the otologist's diagnostic skills and flexibility in technique and prosthesis choice. We review our experience in managing various incudostapedial developmental anomalies discovered during middle ear exploration for suspected otosclerosis in adult patients., Design: A retrospective case-series review was used., Setting: The study was set at a military tertiary referral center., Patients and Other Participants: A review of 185 stapes procedures yielded 8 cases of identified congenital anomalies of the stapes or incudostapedial complex including congenital absence of the oval window with malformed incus and stapes, columellar-type stapes with promontory fixation, stapes superstructure fixation to the fallopian canal, and malformed incus with stapes superstructure fixation to the promontory., Interventions: The developmental anatomy and surgical technique in each case were reviewed. The surgical technique used in each case varied depending on the specific ossicular abnormality found and whether both the stapes and incus were affected., Main Outcome Measures: Comparison of preoperative and postoperative audiometric testing results was performed., Results: All 8 ears (7 patients) that underwent a stapes procedure for either an isolated congenital stapes anomaly or a combined incudostapedial anomaly had a favorable hearing outcome, with 7 ears improving to within 10-dB and 1 ear to within 13-dB air-bone gap on postoperative audiometry., Conclusions: Despite unanticipated findings of congenital anomalies of the stapes and incus during middle ear exploration for conductive hearing loss, a flexible management approach can lead to successful hearing outcomes., (Published by Elsevier Inc.)

Objective: To describe the audiometric results after stapes surgery in a consecutive series of patients with stapes footplate ankylosis combined with another ossicular middle ear anomaly., Study Design: A retrospective analysis of charts collected between 1986 and 2001., Setting: A tertiary referral center., Patients: A total of 25 patients (30 ears) underwent exploratory tympanotomies and ossicular reconstruction., Main Outcome Measure: Audiometric results., Results: Overall, a mean gain in air conduction of 18 dB (from 49 dB to 31 dB) and a mean postoperative air-bone gap (ABG) of 20 dB (mean preoperative ABG, 40 dB) were observed. The ABG closure was 20 dB or less in 70% of cases, which is in agreement with the few results reported in the literature. Moreover, the audiometric results remained stable. In the group of ears with a syndrome, the mean gain of air conduction was only 19 dB, which was comparable to that observed among nonsyndromic ears., Conclusions: Surgery for congenital stapes footplate ankylosis with a concomitant ossicular chain anomaly can provide worthwhile hearing improvement. The ABG closure was 20 dB or less in 21 of 30 ears (70%). Most ears had some sensorineural impairment (10-20 dB), which influenced the final hearing level after surgery. Over recent decades, the technique of the malleostapedotomy procedure has been improved. Preoperative assessment is mandatory for syndromal diagnoses, which might be important for patient counseling and prognosis.