Congenital Ossicular Malformation Research Articles

Spontaneous Regression of Congenital Cholesteatoma: A Report of Two Cases with Differing Ossicle Involvement

The mechanism of congenital cholesteatoma has not been clarified yet. Usually, middle ear cholesteatoma (congenital and acquired) is a progressive and destructive disease that requires surgery for complete cure. In recent years, early detection of congenital cholesteatomas has become more frequent due to advances in endoscopy and microscopy. Along with these developments, there have been reports of congenital cholesteatoma regressing spontaneously, as seen by tympanic and computed tomography findings over time. Herein, we report two cases of spontaneous regression of cholesteatoma, both with ossicular chain malformation. One cholesteatoma was close to the ossicles. In the other case, the cholesteatoma and ossicle were separated; however, the ossicular morphology was inconsistent with that of congenital ossicular malformation. Further similarities and differences between these two cases are discussed herein.

Malleus BarおよびIncus Barを伴った先天性耳小骨奇形

Malleus BarおよびIncus Barを伴った先天性耳小骨奇形

Analyses of the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane

To analyze the clinical characteristics of unilateral conductive hearing loss with intact tympanic membrane, and summarize the key diagnostic points, differential diagnosis and observe the effects of surgical treatment. We reviewed data from 82 patients with unilateral conductive hearing loss with intact tympanic membranes who accepted the exploratory tympanotomy from April 2011 to September 2013. There were 41 males and 41 females, aged from 7 to 66( averaged 26.5±13.7)years, with a history of one month to 50 years. The history, clinical symptoms, audiological evaluation, high resolution temporal bone CT, the results of surgical exploration and hearing reconstruction were analyzed. The exploratory tympanotomy revealed 43 cases of congenital middle ear malformations (52.4%), 22 cases of otosclerosis (26.8%), eight cases of congenital cholesteatoma (9.8%), six cases of trauma induced conductive hearing loss (7.3%), three cases of congenital ossicular malformations with congenital cholesteatoma (3.7%). Progressive hearing loss was common in patients with otosclerosis and congenital cholesteatoma, and patients with congenital middle ear malformations described their hearing loss since childhood. High resolution temporal bone CT of congenital middle ear malformation, trauma induced conductive hearing loss, congenital cholesteatoma diagnosis rate was 40.0%, 50.0%, and 83.3% respectively. The preoperative air-conductive threshold of patients with absence of the oval window were increased to (66.9±1.1)dBHL, the preoperative bone-conductive threshold achieved (28.3±10.4)dBHL at 2 000 Hz. While patients with stapes fixation and that with ossicular chain discontinuity were (27.2±9.7)dBHL and (17.8±8.8)dBHL(P=0.000)respectively. Through the tympanic exploration with endaural incision under the microscope, different hearing reconstruction were applied according to different lesions. After the operation, the hearing level of 52 patients with return visit were improved, the mean air-conductive threshold were decreased from (60.0±11.4)dBHL to (32.2±12.1)dBHL(P=0.000); and the mean ABG were decreased from (43.2±12.0)dB to (16.3±9.4)dB(P=0.000). Congenital middle ear malformations, otosclerosis, congenital cholesteatoma are the most common causes in unilateral conductive hearing loss with an intact tympanic membrane. The diagnosis rate can be improved by analyzing the clinical features. Through exploratory tympanotomy and hearing reconstruction, we can clarify the diagnosis and achieve a satisfying hearing recover.

Analysis of Clinical characteristic of Simple congenital ossicular malformation and Ossicular chain reconstruction

Analysis of Clinical characteristic of Simple congenital ossicular malformation and Ossicular chain reconstruction

Congenital ossicular chain malformations with mobile stapes in children: Results in 17 cases.

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). A nonrandomized, nonblinded, prospective case series. 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. 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. Ossicular reconstruction is a feasible treatment option in children with congenital malformations of the ossicular chain with a mobile stapes footplate. 4. Laryngoscope, 126:682-688, 2016.

中耳臨床]外耳道・鼓膜の正常な先天性伝音性難聴症例の検討

中耳臨床]外耳道・鼓膜の正常な先天性伝音性難聴症例の検討

Congenital Ossicular Malformations in father and daughter

Congenital Ossicular Malformations in father and daughter

Postoperative analysis of congenital ossicular malformation in children

Postoperative analysis of congenital ossicular malformation in children

耳小骨奇形２７耳の検討―聴力改善不成功例を中心に―

Despite otological surgical progress improving clinical congenital ossicular malformation management, some cases remain inadequately treated. We report 27 cases of congenital ossicular malformation, focusing on reasons for remaining or delayed postoperative hearing loss evaluated in 27 congenital ossicular malformation cases in Kyoto Prefecture from 2002 to 2008. Overall success was 93% (25/27) 6 months postoperatively. Two ears had no hearing improvement and three delayed hearing loss 8 to 48 months postoperatively. The first two ears underwent small fenestration stapedotomy with malleus attachment piston, and the other three tympanoplasty type III using an autologous ossicle or total ossicular replacement prosthesis (TORP) as a columella. We discuss problems and solutions using a malleus attachment piston or prosthesis, preoperative audio-and radiological findings, and operative findings including facial nerve anomaly and congenital cholesteatoma.

Isolated Congenital Incus Anomaly

Background and Objectives : A non-progressive and conductive hearing loss with normal eardrum, which has no history of trauma and infection is highly suggestive of a congenital ossicular malformation. Among ossicular anomalies, the most common anomalous ossicle is stapes. However, incus anomaly is sometimes encountered with or without stapes anomaly. The objectives of this study are to describe patterns of incus anomaly, encountered in patients who have a normal eardrum and to analyze their surgical results with special reference to the patterns of incus anomaly. Subjects and Method : We reviewed 26 patients (31 ears) with congenital incus anomaly, which were confirmed through exploratory tympanotomy at Ajou University Hospital from 1994 to 2005. We analyzed temporal bone CT, preoperative and postoperative audiometric findings, and surgical findings. Results : 10 anomalous patterns were detected out of 31 cases of congenital incus anomaly. Incus without its long process was the most common anomalous pattern. In 15 out of 26 cases (57.7%), incus anomaly was associated with stapes anomaly, which was usually unilateral. We could get hearing improvement in 26 out of 31 ears (89.1%). Conclusion : Various anomalous patterns of incus were noted in our series. Incus anomaly was often associated with stapes anomaly, which was usually unilateral. The surgical result of incus anomaly was excellent except the cases without stapes.

Congenital Stapes Anomalies with Normal Eardrum

ObjectivesA non-progressive and conductive hearing loss with normal eardrum, but no history of trauma and infection, is highly suggestive of a congenital ossicular malformation. Among ossicular anomalies, stapes anomaly is the most common. The purpose of this study is to describe patterns of stapes anomaly and to analyze its surgical outcome with special reference to its patterns.MethodsWe conducted a retrospective case review. The subjects comprised 66 patients (76 ears) who were decisively confirmed by the exploratory tympanotomy as congenital stapes anomalies without any anomalies of the tympanic membrane and external auditory canal. The preoperative and postoperative audiological findings, temporal bone computed tomography scan, and operative findings were analyzed.ResultsThere were 16 anomalous patterns of stapes among which footplate fixation was the most common anomaly. These 16 patterns were classified into 4 types according to the status of stapes footplate. Successful hearing gain was achieved in 51 out of 76 ears (67.1%) after surgical treatment.ConclusionFootplate fixation was usually bilateral, whereas stapes anomalies associated with other ossicular anomaly were usually unilateral. The success of the surgical treatment of stapes anomaly might depend on its developmental status of the footplate. Stapes anomalies were detected without any fixed patterns, therefore, it is quite possible to detect a large variety of patterns in future.

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.

Summary A clinical study of tympanoplasty for congenital ossicular malformations

Summary A clinical study of tympanoplasty for congenital ossicular malformations

Reconstruction of the entire ossicular conduction mechanism.

Stapes fixation combined with fixation, absence, or malformation of the malleus-incus complex requires an uncommon surgical reconstruction and offers a unique combination of challenges and hazards. This situation may occur in the presence of severe tympanosclerosis, otosclerosis, congenital ossicular malformations, and revision surgery for either stapedectomy or chronic ear disease. In previous reports, this procedure has been grouped with total ossicular reconstruction without much distinction. However, the challenges unique to this problem deserve special consideration. The present report offers a treatment plan for a group of patients requiring reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate. Retrospective review. Three thousand three hundred fifty (3350) charts of patients requiring total ossicular replacement prostheses (TORPs) were reviewed. Of this group of patients, only 21 of 3350 patients from 1977 to 1999 required TORP placement and removal of the stapes footplate. The patients were followed for an average period of 50 months. Hearing results indicated an overall improvement in the air-bone gap of 10 dB, with 52% achieving an air-bone gap of less than 20 dB. Of the 21 cases, 5 revision surgeries were performed. Three were performed because of a displaced TORP (14.2%). and 2 were performed because of extruded TORPs (9.5%). Reconstruction of the entire ossicular conduction mechanism including removal of the stapes footplate can be successfully achieved with improvement of the air-bone gap of less than 20 dB. Hearing results and extrusion rates are comparable to reported results of TORP placement on a mobile footplate. Successful stapedectomy and simultaneous ossicular chain reconstruction can be performed as a single or staged procedure. Special attention is paid to avoid intrusion of the prosthesis into the vestibule.

The use of malleus allografts in ossiculoplasty.

To assess the functional performance of remodeled malleus allografts in a malleus-footplate assembly in terms of hearing results and mid long-term stability. A retrospective study of 60 consecutive patients who underwent a malleus allograft ossiculoplasty from 1993 until 2000. In all cases the incus and the stapedial arch were missing as the result of cholesteatoma (49), chronic otitis (5), incus necrosis resulting from stapes prosthesis (5), and congenital ossicular malformations (1). In all cases malleus allografts were remodeled to form a malleus-stapes assembly. The audiometric results, using such an ossiculoplasty, revealed an overall median gain of 18.3 dB at 2 months, 22.3 dB at 6 months, and 25 dB 1 year postoperatively on Fletcher frequencies. An air-bone gap closure within 20 dB was achieved in 81% of all cases 1 year postoperatively. No cases of extrusion have been seen in our series. Our findings suggest that malleus allografts are capable of generating good and stable functional results as malleus-stapes assembly.

耳小骨奇形症例の検討

Twenty three patients (26 ears) with congenital ossicular malformations were treated in our department from 1982 through 1993.Malformations of the stapes were the most common, especially fixation of the stapes footplate. Two or more malformations were noted in 10 ears.Of the 15 ears with ossicular chain fixation 11 (73.3%) showed a so-called stiffness curve in the standard audiometric examination. On the other hand, only 4 of the 8 ears (50.0%) with ossicular discontinuity showed a flat hearing curve.Postoperative hearing was improved up to 30dB (JIS standard) or less in 16 ears (61.5%). There was no significant difference in the postoperative hearing between those with ossicular fixation and those with ossicular discontinuity.

Surgical treatment of congenital ossicular malformation in children

Surgical treatment of congenital ossicular malformation in children

鼓膜所見正常な伝音難聴の臨床的観察

Seventeen cases (nineteen ears) of conductive hearing loss with normal tympanic membrane treated were studied. Seventeen cases were as follows: 5 cases (5 ears), incudostapedial joint dislocation; 5 cases (7 ears), congenital ossicular malformation; 6 cases (7 ears) otosclerosis.The postoper ative hearing-gain was 23.8 dB for incudostapedial joint dislocation and 21.4 dB for congenital ossicular malformation and 22.9 dB for otosclerosis.

先天性耳小骨連鎖異常症例について

先天性耳小骨連鎖異常症例について

Congenital ossicular anomalies without malformations of the external ear.

Eighteen ears of congenital ossicular malformation without deformities of the external ear were presented. They were classified into three groups; eight incudostapedial joint separations, three malleus and/or incus fixations, and seven stapes fixations. The surgical correction for the first group was to connect the malleus handle to the stapes using a silicon tube specially designed or a gelfoam wire in the uncustomary way. This group showed the best surgical results with 35.4 dB of the average hearing gain in speech frequencies. In the second group, removal of the fixed part of the ossicles yielded satisfactory results, but mobilization failed to improve the hearing. For the group of stapes fixation, stapedectomy and the gelfoam wire prosthesis were performed. The result was not satisfactory in some cases with the undeveloped oval window. The embryological consideration leads us to the following conjecture; the incudostapedial joint separation results from the failure in build-up of "the secondary continuity", the malleus and/or incus fixation is the result of disturbances in separation process by the undifferentiated mesenchyme, and the stapes fixation is due to maldevelopment of the stapedial lamina. This classification for the congenital ossicular malformation is practically valid as it would suggest the maldeveloped point in ossicular genesis and results of surgical correction.