Congenital middle ear malformation: clinical analysis and discussion of classification

Audiologic results of total ossicular replacement prosthesis bypassing the stapes suprastructure in canal wall down mastoidectomy

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. 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. 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. 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.

Objective OEvaluate the effect of ossicular reconstruction with partial ossicular replacement prostheses (PORP) or toutal ossicular replacement prostheses (TORP). Methods We effectively connected the ossicular chain used the PORP or TORP, replaced the destructive autologous ossicular. Results All the patients were followed-up for 6-47 months, the tympanic membrane got well, the averaged postoperative air conduction reduced 27.8 dB HL, the effectiveness was 79.3%. The recurrent and the reject reaction were zero. Conclusion It is considered that the PORP or TORP was a safety and useful material for ossicular reconstrction in the tympanoplasty. Key words: Otitis media; Middle ear malformation; Artificial ossicle; Tympanoplasty

To analyze the outcomes of exoscopic versus microscopic ossicular chain reconstruction (OCR). Retrospective chart review. Tertiary care otology-neurotology practice. Adult subjects with a diagnosis of ossicular discontinuity from 2018 to 2022. Exoscopic or microscopic primary OCR (without mastoidectomy) with a partial ossicular replacement prosthesis (PORP) or total ossicular replacement prosthesis (TORP). Audiometric outcomes at 3 months and 1 year postoperatively including bone and air pure tone averages (PTA), air-bone gap (ABG), change in ABG, speech reception threshold (SRT), and word recognition score (WRS). Secondary outcomes included operative time and complication rates of primary and delayed graft failure, tympanic membrane lateralization, prosthesis extrusion, cerebrospinal fluid leak, facial nerve injury, profound hearing loss, persistent tinnitus, and persistent vertigo. Sixty ears underwent primary OCR and were subdivided based on prosthesis type (PORP and TORP) and surgical approach (exoscope vs microscope). Exoscopic OCR was performed on 30 ears (21 PORP, 9 TORP), and microscopic OCR was performed on 30 ears (19 PORP, 11 TORP). In the overall group (PORP + TORP) and in the PORP and TORP subgroups, there were no significant differences in 1) demographics, 2) intraoperative findings, and 3) audiometric outcomes of bone and air PTA, ABG, change in ABG, SRT, and WRS at 1 year postoperatively. Operative time was 64.7 and 59.6 minutes for the exoscopic and microscopic group, respectively ( p = 0.4, 95% CI [-16.4, 6.1], Cohen's D = 0.2). Audiometric and surgical outcomes after exoscopic and microscopic OCR are comparable.

To examine hearing results in pediatric patients after ossicular reconstruction with partial ossicular replacement prostheses (PORPs) and total ossicular replacement prostheses (TORPs) in children with chronic otitis media. A retrospective chart review was performed on 55 pediatric patients with chronic otitis media who underwent ossicular reconstruction from 1991 to 1998. Patients' audiograms were evaluated preoperatively and postoperatively for pure-tone average (PTA), air-bone gap (ABG), speech reception threshold (SRT), method of ossicular reconstruction, and management of the mastoid. Twenty-seven patients underwent ossicular reconstruction with TORPs. The average preoperative ABG was 40.1 dB, and the average postoperative ABG was 31.6 dB. Forty-one percent of the children improved their PTA greater than 10 dB postoperatively, and 52% of children did not change their ABG by more than 10 dB postoperatively. Nineteen percent of children with TORPs had a postoperative ABG less than 20 dB, and 44% of children with TORPs had a postoperative ABG less than 30 dB. Twenty-eight patients underwent ossicular reconstruction with PORPs. The average preoperative ABG was 29.7 dB, and the average postoperative ABG was 22.5 dB. Thirty-two percent of patients improved their PTA by greater than 10 dB, while 57% of children with PORPs did not change their ABG by more than 10 dB postoperatively. Forty-three percent of children with PORPs had an ABG of less than 20 dB postoperatively, and 71% of children with PORPs had a postoperative ABG less than or equal to 30 dB. Children who underwent ossicular reconstruction with PORPs had slightly better postoperative hearing than did children with TORPs. Postoperative hearing was essentially unchanged in approximately 55% of both groups. Preoperative hearing levels may be the most important factor determining postoperative hearing in nonstaged surgery for children with chronic otitis media Long-term hearing results in children with single-stage surgery were not as good as those reported in the literature for staged surgery. Severe mucosal disease and eustachian tube dysfunction may contribute to poorer hearing results in children.

Objective: To evaluate and compare postoperative hearing outcomes in patients who have undergone ossicular chain reconstruction using partial and total ossicular replacement prostheses. Study Design: Prospective Non-randomized Clinical study. Setting: Department of Otorhinolaryngology, Shifa International Hospital, Islamabad. Period: April 2024 to April 2025. Methods: Patients undergoing ossiculoplasty were enrolled consecutively and categorized into two groups based on prosthesis used: total ossicular replacement prosthesis (TORP) and partial ossicular replacement prosthesis (PORP). The selection of prosthesis was determined intraoperatively based on extent of ossicular chain damage. All patients were followed post-operatively. Pre-operative and post-operative hearing thresholds were assessed using pure tone audiometry. Results: The TORP group had a higher preoperative air-bone gap (ABG) at 31.77 ± 10.05 dB, compared to the PORP group at 26.31 ± 10.67 dB. After surgery, ABG improved in both groups, reaching 17.54 ± 10.83 dB in the TORP group and 19.69 ± 10.34 dB in the PORP group. Both results met the criteria for successful ossiculoplasty. The analysis within the TORP group showed significant hearing improvement, with a p-value of less than 0.01. This group had a mean ABG gain of 14.23 dB and a large effect size. In contrast, the PORP group showed a moderate improvement of 6.62 dB, with a p-value of 0.05. However, the differences between the two groups were not statistically significant, with a p-value of 0.185 and a Hedges’ g effect size of -0.519 favoring TORP. Conclusion: Both TORP and PORP effectively treat conductive hearing loss. TORP may provide more benefit for patients with severe preoperative hearing loss or significant ossicular damage. Choosing a prosthesis should depend on what is found during the operation instead of past practices.

To explore the efficacy of total and partial ossicular chain reconstruction using titanium implants. Total of 106 patients (107 ears) underwent ossiculoplasty using titanium from June 2008 to January 2011. Eighty-six ears had partial ossicular replacement prosthesis (PORP) and 21 ears had total ossicular replacement prosthesis (TORP). Patient data were collected retrospectively from the database of surgical cases. Patients were assessed during 6 to 30 months postoperatively to establish middle- and long-term hearing results. Average postoperative air-conduction gain and air-bone gap (ABG) were measured at four frequencies: 0.5, 1, 2, and 4 kHz. Postoperative air-bone gap of 20 dB or less than 20 dB was obtained in 83.7% of PORP cases versus 71.4% of TORP cases. All the cases obtained significant postoperative air-conduction gains. The change in air-bone gap (defined as the difference between the means of preoperative and postoperative ABG) was 12.5 dB for PORP patients and 12.9 dB for the TORP patients. There was no statistically significant difference between the TORP and PORP groups. Prosthesis exclusion was observed in one case (0.9%). Prostheses using titanium type could give good functional results and stability with low exclusion rate to patients presenting chronic otitis media.

Objective:To compare the preoperative presentation, intraoperative findings, and postoperative outcomes between middle ear cholesteatoma with tympanosclerosis （MECwTS） and middle ear cholesteatoma without tympanosclerosis （MECw/oTS）, thereby investigating the clinical characteristics of MECwTS. Methods:A retrospective analysis was conducted on the clinical data of 120 patients with middle ear cholesteatoma. Patients were divided into two groups based on the presence or absence of concomitant tympanosclerosis: the MECwTS group （n=49） and the MECw/oTS group （n=71）. All patients underwent preoperative evaluations including temporal bone CT, otoscopic examination, pure-tone audiometry, tympanometry, and assessment using the Zurich Chronic Middle Ear Inventory （ZCMEI-21） quality of life scale. All patients underwent canal wall down mastoidectomy with tympanoplasty. Concurrent ossicular chain reconstruction was performed: partial ossicular replacement prosthesis （PORP） in 83 cases and total ossicular replacement prosthesis （TORP） in 37 cases. Intraoperative disease severity was assessed using the Cholesteatoma Comprehensive Score Scale （CCSS）. Postoperative follow-up lasted at least one year and included pure-tone audiometry, otoscopic examination, and the ZCMEI-21 scale administered at ≥1 year post-surgery. Preoperative and postoperative air-bone gap （ABG） and ZCMEI-21 scores were compared between the MECwTS and MECw/oTS groups. Additionally, surgical efficacy was defined as a postoperative ABG ≤20 dB; the hearing improvement efficacy of PORP versus TORP was compared based on this criterion. Results: ①Preoperative ABG showed no significant difference between the MECw/oTS and MECwTS groups（P>0.05）. Postoperative ABG was （18.65±10.21） dB in the MECw/oTS group versus （22.55±9.53） dB in the MECwTS group, demonstrating a statistically significant intergroup difference （P<0.05）. ②Intraoperative CCSS scores were significantly higher in the MECwTS group （8.04±2.18） compared to the MECw/oTS group （5.93±1.44） （P<0.05）. ③Preoperative ZCMEI-21 scores showed no significant difference between groups （P>0.05）. Postoperative ZCMEI-21 scores were （22.24±8.11） in the MECw/oTS group versus （27.02±7.21） in the MECwTS group, indicating a statistically significant difference （P<0.05）. ④Postoperative ABG ≤20 dB was achieved in 54 patients （65.06%, 54/83） in the PORP group and 16 patients （43.24%, 16/37） in the TORP group. This difference in efficacy rates was statistically significant （P<0.05）. The overall efficacy rate for ossiculoplasty was 58.33% （70/120）. Conclusion: Patients with MECwTS exhibit more severe middle ear and mastoid pathology compared to those with MECw/oTS, resulting in poorer postoperative hearing levels and quality of life outcomes. Both PORP and TORP implantation can improve postoperative hearing to some extent; however, PORP appears to offer superior hearing improvement efficacy compared to TORP.

To assess endoscopic and microscopic ossiculoplasty audiometric outcomes. Retrospective review. Tertiary academic center. Adult patients who underwent ossiculoplasty with either partial ossicular replacement prosthesis (PORP) or total ossicular replacement prosthesis (TORP) from 2010 to 2019 with at least 1 year of audiometric follow-up were included. Endoscopic or microscopic ossiculoplasty. Postoperative air-bone gap (ABG) after at least 1 year. A total of 198 patients, 53.5% female, and a median age of 47.5 years, met inclusion criteria. 64.1% of patients were reconstructed with a PORP, and 31.8% were reconstructed using an endoscopic approach. The median audiometric follow-up was 27 months. The median postoperative ABG was 16.9 dB overall, 15.6 dB for PORP reconstruction, and 19.4 dB for TORP reconstruction (PORP versus TORP, p = 0.002). For TORP reconstructions, the median ABG for both endoscopic and microscopic TORP was 19.4 dB ( p = 0.92). For PORP reconstructions, the median ABG for endoscopic PORP was 12.3 dB compared with 16.3 dB for microscopic PORP ( p = 0.02). Using multivariate linear regression to predict postoperative PORP ABG, and controlling for age, prior ossiculoplasty, middle ear mucosal disease (granulation, fibrosis, polyposis), middle ear atelectasis, myringitis, contralateral middle ear disease, and use of byte prostheses, endoscopic PORP reconstruction was associated with improvement in ABG over the microscopic approach by 4.4 dB ( p = 0.04). For PORP ossiculoplasty procedures, endoscopic ossiculoplasty is associated with improved postoperative ABG compared with microscopic ossiculoplasty.

BackgroundMiddle ear ossiculoplasty refers to the reconstruction of the middle ear ossicular chain when there is an in continuity or fixation of the ossicular chain due to disease process or trauma. Various materials and techniques have been used to regain the function and continuity of the ossicular chain. But the ideal reconstruction materials and techniques are yet to be developed. The first ossiculoplasty was reported in modern medical literature by Matte in 1901. (1) Since then, various people have used different materials and techniques for ossiculoplasty(2).In a Sri Lankan setting, only a few centres of the country perform ossiculoplasty though almost all the Ear nose throat departments in the country perform myringoplasty for CSOM (Chronic suppurative otitis media). The main purpose of myringoplasty is to make the ear safe from recurrent ear infection. Improving hearing is not the main aim for myringoplasty in Sri Lanka though some patients get the hearing benefit.Material and methodGray’s total ossicular replacement prosthesis (TORP )and partial ossicular replacement prosthesis (PORP) were used. Retrospective data analysis was carried out from patients who underwent surgery from 1st of July 2017 for three-year periods and calculated the success rate using the improvement of hearing in postoperative audiograms.Conclusion and recommendationsOur experience in ossiculoplasty with TORP and PORP showed a reasonable success rate on hearing for traumatic or infective conductive hearing loss. We recommend carrying out it routinely with myringoplasty in the case of damage ossicular chain in the middle ear. We also recommend the ministry of health to make available other types of ossicular prostheses like angular clip prosthesis and malleus replacement prosthesis in addition to TORP and PORP.

This study compares hearing outcomes of two prosthesis materials, bone and titanium, used in ossiculoplasty. This retrospective nationwide registry-based study uses data systematically collected by the Swedish Quality Registry for Ear Surgery (SwedEar). The data were obtained from clinics in Sweden that perform ossiculoplasty. Patients who underwent ossiculoplasty using either bone or titanium prostheses were registered in SwedEar between 2013 and 2019. Hearing outcome expressed as air-bone gap (ABG) gain. The study found no differences between bone and titanium for ABG or air conduction (AC) for either partial ossicular replacement prostheses (PORP) or total ossicular replacement prostheses (TORP). In a comparison between PORP and TORP for ABG and AC outcomes, regardless of the material used, PORP showed a small advantage, with an additional improvement of 3.3 dB (95% CI [confidence interval], 0.1-4.4) in ABG and 2.2 dB (95% CI, 1.7-4.8) in AC. In secondary surgery using TORP, titanium produced slightly better results for high-frequency pure tone average. The success rate, a postoperative ABG ≤20 dB, was achieved in 62% of the operations for the whole group. Both bone and titanium used to reconstruct the ossicular chain produce similar hearing outcomes for both PORP and TORP procedures. However, titanium may be a preferable option for secondary surgeries involving TORP. The success rate, a postoperative ABG ≤20 dB, is consistent with other studies, but there is room for improvement in patient selection criteria and surgical techniques.

To compare the results of ossiculoplasty with two different partial ossicular replacement prostheses (PORP) to ossiculoplasty with a total ossicular replacement prosthesis (TORP) in patients with an intact stapes suprastructure. All patients required primary or revision surgery for chronic middle ear disease and ossicular reconstruction with either a PORP or a TORP, as well as a with an intact stapes suprastructure. In total, 141 patients receiving classic tympanoplasty with PORP (N = 92), ossiculoplasty with PORP with ball joint (N = 22), or TORP (N = 27) between January 2011 and March 2017 were included in this study. The inclusion criterion was an intact stapes suprastructure. The underlying indication for surgery was either middle ear disease, such as cholesteatoma, or revision surgery for audiological improvement. The main outcome measures were four-frequency pure tone average (0.5, 1, 2, 3 kHz) at early and late follow-up after ossiculoplasty, the effects of clinical factors including the underlying middle ear disease, and primary or revision surgery. All patients showed a significantly reduced air-bone gap (ABG 0.5, 1, 2, 3 kHz) at late follow-up (mean: 18 dB) compared to preoperative measurements (mean: 25.5 dB). A significantly better outcome in ABG closure was shown among patients with a preoperatively intact tympanic membrane, with an intact stapes suprastructure or without preexisting cholesteatoma. Outcome was not significantly influenced by the prosthesis-type, the creation of an open mastoid cavity, the status of the mucosa, or the indication for surgery. TORP with an intact stapes suprastructure is a safe procedure and provides audiological outcomes similar to PORP. 2C Laryngoscope, 130:768-775, 2020.

Objective: To explore the social and clinical factors that predict audiometric outcomes in patients undergoing ossicular chain reconstruction. Methods: A retrospective analytical cohort study was conducted, including patients 18 years of age or older with a history of chronic otitis media (COM) and/or any of its complications, who underwent ossicular chain reconstruction with Partial Ossicular Replacement Prosthesis (PORP) or Total Ossicular Replacement Prosthesis (TORP), at Hospital San José and Hospital infantile Universitario de San José between 2012 and 2020. We excluded patients with ossicular chain malformations and those with incomplete information. Information about sociodemographic and clinical factors was collected. Additionally, the surgery findings information was analyzed using the Ossiculoplasty outcome parameter staging ( OOPS) index. Results: A total of 35 adult patients who underwent ossicular chain reconstruction were retrospectively studied. An improvement was evidenced in the Preoperative Pure-Tone Average (PTA) and postoperative PTA (p-value=0.036), as well as in the pre and postoperative air-bone gap (ABG) (p-value &lt; 0.01). A moderate correlation coefficient was found between the OOPS index and the postoperative PTA (p= 0.429), and between the OOPS index and the postoperative (ABG) (p= 0.653). Conclusion: We found that a higher OOPS score is correlated with worse hearing outcomes postoperatively, and there was no association between the demographic or pathologic factors with a worse postoperative hearing outcome. Therefore, OOPS index can predict audiometric outcomes in patients undergoing ossicular chain reconstruction in a developing country, regardless of the demographic or pathologic factors.

Classification of facial nerve aberration in congenital malformation of middle ear: Implications for surgery of hearing restoration

Class III Congenital Middle Ear Anomalies: Refining the Teunissen-Cremers Classification Based on Stapes Suprastructure Viability.