Salivary Gland Choristoma of the Middle Ear in a Child: A Case Report.

Salivary gland choristoma is an extremely rare middle ear pathology. We present the case of a 10-year-old girl with unilateral conductive hearing loss. Tympanotomy showed a nonspecific middle ear mass, absence of stapes, anomaly of incus, and displaced facial nerve. It was not possible to remove the mass completely. Histology confirmed salivary gland choristoma. The hearing in this case can be improved with a bone-anchored hearing aid.

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.

Management of Incus Defects in Children: Comparison of Incus Transposition Versus Glass Ionomer Cement.

The aim of this study was to investigate the functional results of glass ionomer cement (GIC) and incus transposition (IT) used for treatment of incus long process defects in pediatric patients. The medical records of 40 patients, aged 10 to 16 years, who underwent ossicular reconstruction due to incus long process defect between January 2010 and July 2017 were reviewed for age and gender, types of ossiculoplasty technique, types of graft used for tympanic membrane repair, preoperative-postoperative audiological examinations, and length of follow-up. The mean preoperative air-bone gap (ABG) was 29.40 (8.47) dB, the mean postoperative ABG was 15.25 (8.55) dB in the entire group, and the difference was statistically significant (P < .001). The functional outcome (ABG ≤ 20 dB) was 80% (32 patients) in the entire group. The mean hearing gain was 18.83 (9.43) dB and postoperative mean ABG was 20 dB or better in 17 (94.4%) patients in the GIC group. The mean hearing gain was 10.31 (9.44) dB, and postoperative mean ABG was 20 dB or better in 14 (63.6%) patients in the IT group. The mean hearing gain was significantly higher in the GIC group than in the IT group (P = .026), and the postoperative mean ABG was significantly higher in the IT group than in the GIC group (P = .045). The functional success rates were significantly higher in GIC group than in IT group (P = .020). There were no statistically significant differences between groups in terms of postoperative speech reception threshold and word recognition scores (P = .628, P = .260, respectively). The graft success rates were 94.4% (17 patients) in the GIC group and 90.9% (20 patients) in the IT groups. There was no statistically significant difference between groups in operated side (P = 1.000). Reconstruction of small incus long process defects using GIC is a valuable technique and can be safely used in pediatric patients. The hearing outcomes are significantly better in GIC rebridging technique when compared with a sculpted IT technique.

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.

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".

Neuroimaging findings in Emanuel Syndrome.

Emanuel syndrome is a rare inherited chromosomal abnormality caused by an unbalanced translocation of chromosomes 11 and 22. Clinically, Emanuel syndrome is characterized by a wide spectrum of congenital anomalies, dysmorphisms, and developmental disability often confused with other similar syndromes. Outside of genetic testing, diagnosis remains challenging and current literature on typical radiologic findings is limited. We present classic neuroimaging findings of Emanuel syndrome consistent with prior literature including microcephaly, microretrognathia, external auditory canal stenosis, and cleft palate; and also introduce the additional maxillofacial anomaly of dysplastic middle ear ossicles, to our knowledge not previously described in the literature. Recognition of findings leading to earlier diagnosis of Emanuel syndrome may improve outcomes and quality of life for patients and their families.

Results of VSB implantation at the short process of the incus in children with ear atresia.

OBJECTIVE(S): To describe a new and more simple surgical procedure for implanting the Vibrant Soundbridge (VSB) on the short process of the incus in children with ear atresia and atretic plate and present the audiometric results. METHODS: Since 2014, pre- and post-operative audiometric tests with tonal and vocal audiometryat the maximal follow up, respectively 33, 22 and 12 months were performed after VSB implantation at the ENT department, Necker Enfants Malades Hospital, Paris, France. 3 children aged 11, 9 and 15 years with conductive hearing loss due to high grade ear atresia and absent ear canal were implanted. The malformed and fused malleus-incus complex was found to be immobile in two of the subjects, who then underwent a superior tympanotomy to carefully remobilize the malleus. Stapes were mobile in all cases. The FMT clip was customized and crimped on the short process, stabilized by the metallic wire. RESULTS: At the maximum follow-up time, the bone conduction was unchanged, and the mean aided ACPTA was 21 dB, 29 dB and 30 dB, compared to 66 dB, 63 dB and 68 dB unaided, respectively. The word recognition score (WRS) at 65 dB SPL increased from 10%, 10% and 70% (unaided) respectively to 100% (aided). CONCLUSIONS: The long process of the incus is usually very hypoplastic in patients with ear atresia with a fused malleus-incus complex lateral to the stapes. VSB coupling to the long process of the incus or the stapes may be challenging in small mastoids. Coupling to the short process of the incus is comparatively a simple procedure, since there is no need for posterior tympanotomy and has no proximity to the facial nerve. The results of this study are similar to other ear atresia cases in the literature with classic FMT placement on the stapes or long process of the incus. Outcomes should be confirmed in a larger number of patients.

Congenital anomalies of the incudostapedial joint.

OBJECTIVE: To describe congenital anomalies of the incudostapedial joint (ISJ) and to discuss the possible unique embryogenesis of the ISJ based on the two anomalies that were encountered. SETTING: Tertiary care medical center. SUBJECTS AND METHODS: Retrospective review of the medical records of all patients with ISJ anomalies. RESULTS: Four patients presented with congenital hearing loss. Upon further workup, we observed a preserved incudostapedial joint (ISJ) with deficiencies of the incus and stapes in three cases. Our fourth case demonstrated the inverse pattern of the congenital anomalies in which the ISJ was missing with an intact proximal incus and stapes crura. Three patients opted for surgical intervention with improvement in hearing. One case preferred hearing amplification over surgery. CONCLUSION: Isolated ISJ malformations are uncommon potential causes of congenital conductive hearing loss. Although numerous patterns of ossicular anomalies have been reported in the literature, our case series is the first to demonstrate both the absence of the ISJ in one patient and the presence of the ISJ in the presence of missing stapes crura and incus body in other patients. Though limited by the small number of cases, the inverse relationship of the single case compared to the three other cases, suggests a possible independent embryological development pathway for the ISJ. Therefore, an embryological explanation of the defects should be considered. Additionally, surgical intervention can improve hearing outcomes for patients with isolated ISJ anomalies.

The "boomerang" malleus-incus complex in congenital aural atresia.

"Boomerang" malleus-incus fusion deformity is identified on axial high-resolution CT in a subset of patients with congenital aural atresia, and it is associated with an absent incudostapedial joint and stapes capitulum and attachment of the incus to the tympanic segment of the facial nerve canal. Twelve patients with this deformity were identified on a retrospective review of imaging from a cohort of 673 patients with congenital aural atresia, with surgical confirmation in 9 of these patients. Eight of 9 patients underwent partial ossicular replacement prosthesis reconstruction with improvement in hearing outcome. We hypothesize that the boomerang anomaly represents a more severe ossicular anomaly than is normally seen in congenital aural atresia, arising from an arrest earlier in the embryonic development of the first and second branchial arch. This has potentially important implications for surgical planning because hearing outcomes with placement of prosthesis may not be as good as with conventional atresia surgery, in which reconstruction is performed with the patient's native ossicular chain.

Congenital incus fixation to the fallopian canal.

IMPORTANCE: An isolated congenital anomaly of the ossicular chain is a rare finding in an ear that, otherwise, appears clinically normal. The most common congenital ossicular anomaly is fixation of the stapes footplate. The least common congenital ossicular anomaly is isolated incus fixation, with only a few reported in the literature. OBSERVATIONS: We describe a woman in her 50s with a long history of left-sided hearing loss and unremarkable neurotologic findings aside from a tuning-fork examination result suggestive of left-sided conductive hearing loss. Intraoperatively, she was found to have isolated fixation of the incus to the fallopian canal. The incus-fallopian canal fixation was separated, and an autologous bone graft was placed between the lenticular process and stapes capitulum to create elevation and prevent refixation. One month postoperatively, an audiogram revealed a nearly complete air-bone gap closure. CONCLUSIONS AND RELEVANCE: Patients who present with conductive hearing loss, normal physical examination findings, and an apparent normal radiograph are generally assumed to have otosclerosis. This case illustrates an unanticipated unique anomaly that was surgically corrected by releasing the bony fixation of the incus to the fallopian canal and placing an autologous bone graft at the incostapedial joint.

Fibrous incudostapedial joint in congenital aural atresia.

OBJECTIVES: (1) Determine the prevalence of a non-bony or fibrous incudostapedial (IS) joint in the setting of congenital aural atresia. (2) Assess this anomaly's impact on surgical management and associated hearing outcomes. STUDY DESIGN: Retrospective chart review. SETTING: Subspecialty private practice. SUBJECTS AND METHODS: Operative reports and audiometric data of patients who underwent congenital aural atresia repair by a single surgeon from 2007 to 2011 were reviewed for operative anatomic findings and audiometric outcomes. RESULTS: Two hundred twenty-eight operations on 206 ears were performed. Median age was 5 years old. Fifty-five (26.7%) of these ears had a fibrous IS joint. The severity of this anomaly was graded as mild in 23 ears, moderate in 20 ears, and severe in 12 ears. Mean postoperative pure tone air conduction (PTA2) in the severely fibrous group was 51 compared to 46 in the moderate group (P = .03) and 41 in the mild group (P = .006). Patients with a fibrous IS joint who underwent successful ossicular chain reconstruction (OCR) had a mean postoperative PTA2 of 30, which was a significantly better outcome than in patients with moderately or severely fibrous IS joints who did not have OCR (P < .05). CONCLUSION: A fibrous IS joint was seen in 27% of patients undergoing repair of congenital aural atresia. The severity of this anomaly has important implications for postoperative hearing results. These findings suggest that ossicular chain reconstruction should be performed in moderately or severely fibrous cases.

The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia.

CONCLUSION: The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia (CAA). OBJECTIVE: To analyze anatomic variations observed on high-resolution temporal bone computed tomography (TBCT) in patients with CAA, the extent of inferior displacement of the mastoid tegmen and the size of the incudo-stapedial (IS) joint angle were compared with surgical parameters for atresiaplasty, such as Jahrsdoerfer score and hearing acuity. METHODS: Sixty-one patients with unilateral CAA underwent high-resolution TBCT and hearing tests. We evaluated TBCTs in terms of Jahrsdoerfer criteria and analyzed the relationships among the inferior displacement of the mastoid tegmen, size of the IS joint angle, hearing acuity, and the Jahrsdoerfer score on the atretic side. RESULTS: IS joint angle on the atretic side was found to be 120.6 ± 11°, which was significantly greater than the corresponding value of 103.4 ± 5.4° on the normal side. Cholesteatoma occurred significantly more often in CAA patients with narrow external auditory canals (EACs) (9/27; 33.3%), compared with CAA patients with no EAC opening (2/34; 5.9%). There was a significant relation between the extent of inferior displacement of the mastoid tegmen (28.9% in the atretic side and 17.3% in the normal side) and the Jahrsdoerfer score (p < 0.0001).

Can the pathogenesis of auditory ossicular malformations be explained by the branchial-based theory?--Evaluation by the consecutive distribution of embryologic foci in 87 cases.

BACKGROUND: Development of the auditory ossicles is initiated by induction of the cartilage primordium of each ossicle between the fifth and seventh fetal week. It is well known that primordium of the upper part of the ossicular chain is derived from the first branchial arch and that of the lower part develops from the second branchial arch. Previous studies have suggested that auditory ossicular malformations are caused by deficiencies in the early period of induction of the cartilage primordium. However, auditory ossicular malformations can occur at any developing stage, and their clinical features are very complicated. OBJECTIVE: The precise embryologic foci of auditory ossicular malformations were estimated by evaluating the consecutive distribution of these anomalies, and the pathogenesis of these malformations was discussed using new aspects. STUDY DESIGN: Retrospective study. PATIENTS: Eighty-seven ears of 78 patients that underwent surgical treatment after the diagnosis of an auditory ossicular malformation in Niigata University Hospital between 1998 and 2012. MAIN OUTCOME MEASURES: The types of malformations were roughly divided into 4 groups based on intraoperative findings: Type A--ankylosis of the malleus head or short process of the incus; type B--defect in the connection between the incus and stapes; type C--fixation of the footplate; and type D--a complex lesion combining types B and C. Additionally, the consecutive points of the malformation were precisely evaluated, and a distribution map of the malformation was made. Foci of the malformations of each type were then estimated. RESULTS: Type A malformations were observed in 8 ears, type B in 33 ears, type C in 32 ears, type D in 6 ears, and unclassified anomalies in 8 ears. A deformity was observed in the malleus handle, which is located in the lower part of the ossicle, in 5 of 7 ears with type A malformations, which suggests that the pathogenesis of ankylosis of the malleus head or short process of the incus could not simply be explained by the branchial-based theory. The focus of the type B malformation was located on the long process of the incus and not onthe I-S joint. We suggest that pathogenesis of the defect inthe connection between the incus and stapes could not be explained by a conjugation deficiency in the cartilage primordium but could be explained by an ossification deficiency after the conjugation period. The focus of the location of the defect was shifted more medially in type D malformations than in type B malformations, and this was significantly different, which suggests that this type of malformation is not caused by 2 independent anomalies but is inducted by a monogenic abnormality. CONCLUSION: The foci of auditory ossicular malformations were highly variable, which suggests that the pathogenesis of these malformations could not be simply explained by a branchial-based theory. Auditory ossicular malformations occur at various developmental stages of the auditory ossicles.

Middle ear malformations in identical twins.

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.

Management of incus long process defects: incus interposition versus incudostapedial rebridging with bone cement.

OBJECTIVE: This study aimed to compare the hearing results of incus interposition and bone cement ossiculoplasty in patients with incus long process defects. MATERIALS AND METHODS: Ninety-nine patients with incus long process defects were included. Incus interposition was performed in 49 patients (group 1) and bone cement ossiculoplasty was performed in 50 patients (group 2). Group 1 included 29 female and 20 male patients, with a mean age ± standard deviation of 29.43 ± 12.5 years (range, 8­58 years). Group 2 comprised 32 female and 18 male patients, with a mean age ± standard deviation of 29.1 ± 14.89 years (range, 8­67 years). RESULTS: The mean hearing gain ± standard deviation was 15.2 ± 9.01 dB in group 1 and 19.36 ± 9.08 dB in group 2. Hearing gain was significantly greater in the bone cement group than in the incus interposition group (p = 0.0186). Successful hearing results (i.e. air­bone gap < 20 dB) were achieved by 63.2 per cent of group 1 patients and 78 per cent of group 2 patients. CONCLUSION: Incus interposition and bone cement ossiculoplasty are safe and reliable methods with which to manage incus long process defects. Bone cement ossiculoplasty gives a greater hearing gain in appropriate cases.

The incudostapedial articulation in Down's syndrome (trisomy 21): a temporal bone study.

HYPOTHESIS: Anomaly in the incudostapedial articulation (IS) may be responsible for persistent conductive hearing loss (HL) in Down's syndrome (DS) patients. Our goal was to perform a detailed histopathologic assessment of the role of the IS joint in the conductive HL. BACKGROUND: In addition to other disabilities, 38% to 78% of patients with Down's syndrome (Trisomy 21) have CHL, some of which are persistent despite maximum treatment. The cause of the persistent conductive HL remains unclear. METHODS: Retrospective analysis of temporal bone histopathology. Analyses of the midsection through the IS joint of 21 temporal bones (13 subjects) patients with DS and 31 temporal bones (18 subjects) from healthy subjects, performed by 2 blinded authors. The length of 7 different parameters of the width of the IS joint was measured and compared between the 2 groups. RESULTS: The IS joint of patients with DS was significantly wider than the normal control. As opposed to the control, it did not decrease because of age. CONCLUSION: The persistent conductive HL in some patients with DS might be caused by anomaly of the IS joint. In severe cases, corrective surgery should be considered.

Diagnostic value of bent-lever planes in detecting abnormality of the malleus-incus complex.

OBJECTIVE: To retrospectively investigate the diagnostic value of the bent-lever planes in detecting abnormality of the malleus-incus complex. STUDY DESIGN: Diagnostic test assessment. SETTING: The study was conducted at the Shandong Medical Imaging Research Institute, Shandong University, Jinan, People's Republic of China. SUBJECTS: Eighty-five ears in 74 patients with surgically proved abnormality of the malleus-incus complex. MATERIALS AND METHODS: The bent-lever planes and direct axial images were reviewed by 2 radiologists. The radiologists assigned a value of abnormality or continuity to different parts of the malleus-incus complex. Differences in categorical data were evaluated with the McNemar test. The sensitivity, specificity, Youden index, and interobserver agreement were calculated. RESULTS: There was no significant difference between bent-lever planes and direct axial images in identifying abnormality of the malleus-incus complex. The sensitivity ranged from 71.4% to 97.1%, and specificity ranged from 89.5% to 100.0%. The Youden index ranged from 0.609 to 0.971. The radiologists had almost perfect or substantial agreement in identifying the abnormality of different parts of the malleus-incus complex. CONCLUSION: The bent-lever planes do not add additional diagnostic value for experienced observers.

Ear and further anatomic anomalies in children undergoing stapedotomy.

PURPOSE: Aim of this study was to examine any stapes fixation-related clinical, intraoperative and high-resolution computed tomography (HRCT) findings in children undergoing stapedotomy. PROCEDURES: Detailed diagnostic assessment was performed in 12 children (18 ears) who underwent stapedotomy within the period 2005-2008. RESULTS: Ear malformations such as malleus-incus anomalies, malleus-epitympanum fixation and absence of the stapedius tendon, small external auditory canal and auricle malformations were clinically and/or intraoperatively diagnosed. HRCT identified a petrous high jugular bulb and malformed inner auditory canal in 4 ears; however, it failed to recognize ossicular fixation. Systemic anomalies, namely kyphoscoliosis, esophageal atresia and finger malformations were identified in 2 children. Summing up, additional anatomic anomalies were found in 8 patients (66.7%). CONCLUSIONS: Ear malformations may coexist in children with stapes fixation. Ossicular fixation is not easily recognized with HRCT, and therefore, ossicular mobility should always be tested intraoperatively. In a few cases, pediatric stapes fixation can coexist with systemic dysplasias; in such patients, additional referral to geneticists is recommended.

Hereditary isolated ossicular anomalies in two generations of patients.

We present herein a report of an isolated form of ossicular anomaly that affected two generations of patients. Two female patients, a mother and daughter, were admitted with complaints of conductive hearing loss, with no other anomalies and no history of ear infection. Surgical exploration revealed identical ossicular anomalies: the complete absence of the long process of the incus and fixation of the stapes. This anomaly can be considered to have been inherited in an autosomal-dominant or X-linked-dominant manner. To date, two reports have described isolated forms of congenital ossicular anomalies. Our findings suggest that isolated congenital anomalies can be inherited.