Congenital incudostapedial anomalies in adult stapes surgery: a case-series review.

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.

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.

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.

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.

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.

Unilateral isolated incus agenesis: detection by virtual endoscopy.

We report the first case of unilateral incus agenesis in the literature. Preoperative diagnosis by virtual endoscopy further enhances the significance of this case. We present thin-section temporal bone computed tomographic images and 3-dimensional reconstructed views which were all correlated with intraoperative findings. The patient was successfully treated with an ossicular prosthesis replacement.

Involvement of the incudostapedial joint anomaly in conductive deafness.

CONCLUSION: The outcome of surgery depends on complexities of middle ear anomalies and definite diagnosis can only be achieved during exploratory tympanotomy. We must be aware that the pathology of the congenital ossicular anomalies is variable and careful surgery is needed for hearing improvement. OBJECTIVE: This study aimed to investigate congenital ossicular malformation. SUBJECTS AND METHODS: Fifteen cases of ossicular anomalies without external ear malformation were studied. The anomaly of the incus-stapes complex was the most frequent. There were two cases of fused incudostapedial (IS) joint, which is an extremely rare occurrence. Case 1 was a 33-year-old man, in whom otosclerosis was suspected and exploratory tympanotomy was performed. The IS joint was fused, and the stapes was immobile. Small fenestra stapedectomy was performed. In case 2, a 52-year-old woman, otosclerosis was also suspected and exploratory tympanotomy was performed. The IS joint was fused and the incus was fixed. After the long process of the incus was cut, the stapes became mobile. However, since the incus remained immobile, it was removed and was placed on the stapes. In both cases, the hearing improved after surgery. The averaged hearing gain of 15 cases was 28.8 dB.

Bony sheathed chorda tympani: a unique case of incudomalleolar fixation.

OBJECTIVE: To describe a unilateral conductive hearing loss caused by restriction of the movements of malleus and incus due to chorda tympani in a bony sheath. PATIENT: A 23-year-old man with a stable mild conductive hearing loss in the left ear and mild sensorineural hearing loss in the right ear. INTERVENTION: An exploratory tympanotomy was performed. MAIN OUTCOME MEASURE: Immobility of the ossicles due to a bony structure, which was found to be chorda tympani encircled in a bony sheath. Ossicular system regained its mobility with the severance of the chorda tympani. RESULTS: Hearing was improved both subjectively and audiographically. CONCLUSION: This is the second case of chorda tympani with a bony sheath and the first case of this pathologic finding affecting both malleus and incus. Bony sheathed chorda tympani is considered to be a congenital anomaly of the chorda tympani that causes incudomalleolar fixation and behaves as posterior bony bars.

Conductive deafness with normal eardrum: absence of the long process of the incus.

CONCLUSIONS: The most likely cause of lesions with an absence of the long process of the incus was congenital origin because many cases were associated with the stapedial anomaly. However, the possibility of a potential inflammation of this lesion could not be completely excluded, especially in cases with only an absence of the long process of the incus. OBJECTIVE: To clarify whether the absence of the long process of the incus is caused by congenital anomaly or a potential inflammation. SUBJECTS AND METHODS: A total of 21 patients (5 bilateral cases, 16 unilateral cases) with absence of the long process of the incus were reviewed. Operative and histopathological findings of the removed incus were analyzed. RESULTS: The 21 cases with absence of the long process of the incus were classified into 6 types. Thirteen cases showed the absence of the long process associated with the stapedial anomaly, which were all unilateral. Eight cases showed no association with the stapedial anomaly, among which five cases were bilateral. Three of eight cases not associated with the stapedial anomaly showed evidence of resorption in the incus body such as fibrosis and Haversian canal widening.

[Stapes surgery performed for congenital stapes foot plate fixation--comparison of prosthesis fixation to the malleus vs. the incus].

We report the surgical results of stapes surgery using the Schukneht-type wire piston prosthesis performed on 30 ears with fixation of the stapes footplate and absence of the long process of the incus. The prosthesis was reformed to avoid dislocation and fixed to the handle of the malleus. The surgical results in another 49 ears, the comparator group, in which the prosthesis was fixed to the long process of the incus because the anomaly was restricted to fixation of the footplate without other associated anomalies, were also analyzed for comparison. The mean postoperative air conduction hearing levels were 28.6dB in the subjects and 21.6dB in the comparator group, and the mean hearing improvements were 35.7dB and 29.7dB, respectively. The success rate of the operation, based on the criteria established by the Japan Otological Society, was 90% in the subjects and 98% in the comparator group. The mean postoperative air-bone gap in the subjects was 15.8dB, which was 4.3dB higher than that in the comparator group. The difference between the two groups was considered to be due to the difference in the effectiveness of the conduction mechanism after the surgery. We therefore concluded that the surgical procedure using the Schukneht-type wire piston prosthesis to fix the malleus handle is a useful surgical method that yields satisfactory results.

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.

Malleus Teflon piston prosthesis.

The malleus Teflon piston prosthesis is very useful to reconstruct the sound conducting mechanism of the middle ear in absent or inadequate incus, congenital malformation of the middle ear, and epitympanic fixation of the incus and/or malleus. It has a thin-walled loop at its upper end to lessen the tendency for ulceration of the overlying drum with an inside diameter of 1 mm to fit around the mid portion of the handle of the malleus at an angle of 30 degrees to the shaft of the prosthesis. There is a cut in the distal part of the loop through which it is snapped onto the handle of the malleus after it is separated from the drum. Examples of the various situations in which this prosthesis has been used will be shown together with the results regarding hearing gain, complications, and other problems. Those situations in which the prosthesis was used in 1972 will be reviewed to give a 10-year follow-up, plus some notable examples of other uses of the prosthesis from other times.

Congenital absence of the oval window.

From 1983 to 1987, seven patients with conductive hearing loss were found to have congenital absence of the oval window. Six of these patients underwent vestibulotomies and reconstruction with House wires or total ossicular reconstruction prostheses. Surgical findings included absent or rudimentary stapes in all cases, abnormal facial nerves in four cases, and abnormal long processes of the incus in four cases. All patients had normal external ear canals and tympanic membranes. Hearing initially improved 20 to 45 dB in four of the six patients reconstructed. Over time, however, much of this initial hearing gain was lost. A marked difference in hearing results was evident between this series of absent oval window cases and a recent series of major atresia cases.

Technical refinements and precautions during ionomeric cement reconstruction of incus erosion during revision stapedectomy.

OBJECTIVE: The study describes the technical precautions and short-term hearing results of fast-setting ionomeric cement (SerenoCem) for managing incus erosion in revision stapedectomy. STUDY DESIGN: Observational and retrospective chart review. METHODS: Consecutive patients undergoing ionomeric cement incus reconstruction during revision stapedectomy had surgery on an ambulatory basis in a tertiary care referral center. Main outcome measures included technical details, precautions, and recommendations for handling this new material and 6-week hearing outcomes comparing preoperative and postoperative air-conduction and bone-conduction thresholds. RESULTS: A small amount of ionomeric cement on the tip of otological picks applied to the incus remnant successfully reconstitutes the original length of the long process of the incus. In revision stapedectomy, a crimp-on prosthesis may be placed on the cement-lengthened incus. Six-week postoperative audiograms demonstrated significant closure of the air-bone gap in operated cases. Our experience in a failed case leads us to recommend that the setting time for the cement be increased to no less than 20 minutes as opposed to the manufacturer's recommendation of 10 minutes. Also, revision stapedectomy was more likely to be successful when the prosthesis was placed to the incus remnant and stabilized with cement, rather than placing the prosthesis on the cement itself. CONCLUSIONS: Ionomeric cement permits direct reconstruction of a pathologically shortened incus in revision stapedectomy. Surgeons must be aware of precaution and limitations of this new material. Preliminary results indicate significant hearing improvement with this technique when appropriate precautions are taken.

Congenital absence of the long process of the incus.

OBJECTIVE/HYPOTHESIS: To review the case reports of three generations of one family, suffering from bilateral conductive hearing loss due to congenital absence of the long process of the incus. STUDY DESIGN: Review of the literature regarding hereditary congenital absence of the long process of the incus to determine its mode of inheritance such as autosomal dominant or X-linked. Also document the types and results of surgical repairs performed by previous investigators of this defect and compare them with the present study. METHODS: Three female patients of direct lineage. Mother, daughter, and granddaughter, who were personally seen, operated on, and followed up over a period of 26 years. RESULTS: All three female patients presented with similar symptoms and findings of hearing impairment since birth, with no history of ear infections, and with normal appearing tympanic membranes. However, they had severe conductive hearing loss with normal bone conduction and discrimination. Operative findings were identical in all three patients, with the incus ending in a blind stub, and with absence of the capitulum of the stapes. The type of ossicular reconstruction varied with the changing of times and advances in otologic surgery, as well as the prosthetic materials used. All three patients received improvement in their hearing, and this improvement has been maintained over the 26-year span of the study. CONCLUSIONS: Hearing loss due to congenital absence of the incus is uncommon but not rare. Interestingly, no other instance of inherited cases were reported in the literature. In this family the defect appears to be an inherited trait either due to an autosomal dominant mutation or an X-linked dominant inheritance. This abnormality is amendable to surgery, and any well-executed technique usually gives good hearing results.

Anomalously coursing facial nerves above and below the oval window: three case reports.

Anomalous coursing of a dehiscent horizontal segment of the facial nerve, overlying or running below the oval window, is a rare phenomenon exclusively associated with conductive deafness caused by developmental abnormalities of the stapes and occasionally coincident with additional types of congenital auricular and nonauricular abnormalities. This article describes three new cases of this entity, including one previously unreported coincidence of an abnormally contracted tympanic ring, which should serve to alert the surgeon to the danger of surgical damage to a dehiscent, displaced facial nerve.