Congenital Middle Ear Malformations Research Articles

Clinical features of congenitally enlarged bony portion of Eustachian tube

Objective:To investigate the clinical features of patients with congenitally enlarged bony portions of the Eustachian tube（ET）. Methods:The medical history, physical examination, hearing test, temporal bone high resolution computed tomography（HRCT） of six patients（nine ears） with congenitally enlarged bony portion of the ET were retrospectively analyzed. Results:Four patients were men and two were women. The minimum, maximum, and average ages were 5, 21, and（14.7±6.4） years, respectively. Three malformations were bilateral and three were left-sided. Three ears had conductive hearing loss（average bone and air conduction thresholds were 13.7 dB and 71.3 dB）, three had mixed hearing loss（average bone and air conduction thresholds were 27.7 dB and 83.7 dB）, and one had extremely severe sensorineural hearing loss. The average maximum length and width of the enlarged bony ET on temporal bone HRCT were（22.61±2.94） mm and（6.50±2.33） mm, respectively. The enlargement was combined with an external auditory canal malformation in six ears, narrow tympanic cavity in six, tympanic antrum malformation in five, ossicular chain malformation in seven, cochlear malformation in six, helicotrema malformation in three, vestibule widening in two, semicircular canal malformation in three, vestibular window malformation in six, facial nerve abnormality in five, internal auditory meatus malformation in two, low middle cranial fossa in eight, and severe internal carotid artery malformation in one. Conclusion:Bony ET enlargement is a rare congenital middle ear malformation which could combined with other ear malformations. Patients can have no ET dysfunction but different patterns of hearing loss. The defect is usually found unintentionally during imaging, and the HRCT of temporal bone is significant.

Expert consensus on the application of bone-conducted hearing device in patients with congenital middle and external ear malformations

Expert consensus on the application of bone-conducted hearing device in patients with congenital middle and external ear malformations

Transcanal endoscopic management of isolated congenital middle ear malformations

Background A few studies have reported transcanal endoscopic management of isolated congenital middle ear malformations (CMEMs). Objective The purpose of this study is to describe our surgical experience in endoscopic ear surgery for isolated CMEMs and evaluate the surgical effect of hearing reconstruction. Methods From January 2017 to January 2022, a retrospective study was performed on 36 patients (37 ears) with isolated CMEMs who all underwent endoscopic surgery. Demographic data, high-resolution computed tomography (HRCT) findings, intraoperative findings, surgical management and audiometric data were recorded. Results Anomalies were categorized according to the Teunissen and Cremers classification system: 8 ears were categorized as class I, 8 ears as class II, 19 ears as class III and 2 ears as class IV. The air conduction pure tone average (AC-PTA) of 37 cases was 61.5 ± 8.6 dB preoperatively and 29.6 ± 6.9 dB postoperatively (p < 0.001). The mean preoperative air-bone gap (ABG) significantly decreased from 43.1 ± 8.7 dB to 12.8 ± 5.5 dB postoperatively. 36 of 37 cases (97%) met the criteria for successful operation. Conclusion Isolated CMEMs are mainly manifested as aplasia of the stapes’ superstructure and dysplasia of the long process of the incus. Transcanal endoscopic surgery seems a safe technique for the management of isolated CMEMs.

Multislice Spiral Computer Tomography Findings of Simple Congenital Middle Ear Malformations.

Background Simple congenital middle ear malformations (SCMEMs) are a group of congenital ear malformations. The study aims to analyze the multi-slice spiral computer tomography (MSCT) manifestations of normal ears and SCMEM ears. Objectives This study aimed to investigate the MSCT manifestations of normal ears and SCMEM ears and to evaluate the relationship between the SCMEM and the tympanic segment of the FNC pathway. Methods This was a retrospective case-control study. Patients who were diagnosed with SCMEM were included in the SCMEM group. Patients with vertigo, pulsatile tinnitus, or other symptoms were included in the control group. MSCT examination and image processing of the ossicular chain, facial nerve canal, and related structures were performed and compared between the two groups. Results There were 94 cases in the SCMEM group (125 ears) and 97 cases in the control group (190 ears). Sixty-three cases (67.0%) were unilateral malformations (36 right ears and 27 left ears). MSCT showed congenital stapes malformation in 107 ears (85.6%) and incus long process malformation in 84 ears (67.2%). Among these, simple stapes malformations were found in 41 ears (32.8%), simple incus malformation in 18 ears (14.4%), and stapes malformation complicated with incus malformation in 66 ears (52.8%). The distance between the facial nerve and lateral semicircular canal (FNC-LSC) in the SCMEM group was 1.30 ± 0.64 mm compared to 0.79 ± 0.11 mm in the control group (P < 0.001), and the distance between facial nerve canal and oval window (FNC-OW) was 0.67 ± 0.53 mm in the SCMEM group and 1.13 ± 0.18 mm in the control group (P < 0.001). Conclusion SCMEM occurred mainly in ossicular chain abnormalities. MSCT clearly showed the slight structural changes in the middle ear and provided an accurate basis for preoperative diagnosis.

Right Incus Osteoma in a Child: A Differential Diagnosis of Middle Ear Malformations.

Osteomas of the temporal bone, especially those involving the incus, are rare, unilateral, benign osseous tumors. The clinical presentation is usually isolated conductive hearing loss, and the diagnosis is confirmed by a temporal computed tomography scan. Osteomas of the incus represent a differential diagnosis of congenital middle ear malformations in children, which are the most frequent cause of conductive hearing loss with a normal eardrum in the pediatric population. In case of disabling symptomatology, surgery seems to be a safe way to recover normal hearing.

Effect of malleostapedotomy procedure on 17 cases of congenital middle ear malformation

Objective: To conclude the clinical features and the postoperative efficacy of congenital middle ear malformation treated with Malleostapedotomy (MS), and to explore the security and effectiveness of MS surgery. Methods: The clinical data of 17 patients (18 ears) with congenital middle ear malformation undergoing MS procedure were analyzed. There were 10 males (11 ears) and 7 females (7 ears), aged from 7 to 48 years. The imaging examination, pure-tone audiometry, intraoperative findings and postoperative hearing improvement of these patients were analyzed and summarized, and software SPSS23.0 was used for statistical analysis. Rusults All the 17 patients (18 ears) presented with hearing loss since childhood on the affected sides. Preoperative high resolution CT (HRCT) of the temporal bone revealed definite malformations in 9 ears (6 ears with incus long process dysplasia and 3 ears with anterior and posterior crus dysplasia). Before surgery, the mean bone conductive hearing threshold at 500, 1 000, 2 000 and 4 000 Hz was (15.6±10.2) dB HL, the mean air conductive hearing threshold was (60.6±9.7) dB HL, and the mean air-bone gap was (45.0±8.9) dB. During the surgery, all 18 ears were found to be accompanied by absence or hypoplasia of incus long process. 12 ears had stapes fixation, 6 ears had oval window atresia. All patients were treated with MS procedure by using Piston. The patients were followed up for 3 months to 1 year. The mean bone conductive hearing threshold was (14.7±8.8) dB HL. The mean air conductive hearing threshold was (37.7±11.6) dB HL, and the mean air-bone gap was (23.0±8.0) dB. There were statistically significant differences in the mean air conductive hearing threshold and mean air-bone gap before and after surgery (P<0.05). While there were no statistically significant differences in the mean bone conductive hearing threshold before and after surgery (P=0.550). Conclusions: MS procedure is safe and reliable in patients with congenital middle ear malformation of incus long process dysplasia, stapes fixation or oval window atresia. HRCT is useful in evaluating the major deformity of ossicular chain and facial nerve deformity. However, it is not enough to evaluate the joint of incus-stapes and oval window atresia. MS surgery in middle ear malformation requires advanced surgical experience and skills. The hearing improvement can be significant, even though some air-bone gap after surgery exist.

Selection and application in the approach of Bonebridge implantation for bilateral congenital malformation of external and middle ear: selection and application

Objective:According to whether auricle reconstruction has been performed before the implantation of Bonebridge, the different surgical plan of combination of ear reconstruction and hearing rehabilitation with Bonebridge were respectively applied for the individuals with congenital outer and middle ear malformation. The study aim to explore the feasibility of personalized comprehensive treatment of congenital outer and middle ear malformation. Methods:We developed individualized surgical plans of Bonebridge implantation and auricular reconstruction for 35 patients with bilateral external and middle ear malformation. Six patients underwent Bonebridge implantation on one side, and the post-auricular skin expander implantation on the other sidesimultaneously; seven patients underwent Bonebridge implantation at the same time as the second stage of auricular reconstruction; twenty-two patients had their Bonebridge implantations performed after the reconstruction of the auricles. Results:No intraoperative complications occured in 35 patients. No facial paralysis, vertigo, tinnitus and cerebrospinal fluid leakage was reported. One patient had skin infection after Bonebridge implantation. The Bonebridge was removed and 7 months later and the Bonebridge implantation was re-performed on the same side. No complication occurred after 16 months of follow-up. Conclusion:According to the individual condition of the patients, different surgical plans of Bonebridge implantation and auricular reconstruction can be selected personally, which is beneficial to obtain the ideal aesthetic and hearing outcome.

Isolated congenital middle ear malformations: comparison of preoperative HRCT and surgical findings

Objective:To compare preoperative temporal bone HRCT readings to intraoperative findings during exploratory tympanotomy for suspected cases of isolated congenital middle ear malformations（CMEMs） and summarize the malformations that can be diagnosed with HRCT. Method:A retrospective study was conducted. All cases were confirmed as isolated CMEMs during surgery. Detailed clinical records were reviewed, with a focus on imaging and surgical findings. Result:One hundred and fifty-two patients and 168 ears were reviewed. One hundred and seven cases（63.7%） could be identified as isolated CMEMs and at least one as middle ear anomaly using preoperative HRCT. Sixty-one cases（36.3%） were reported to be completely normal and the patients underwent exploratory tympanotomy to determine the final diagnosis. Stapes fixation, either alone or associated with other ossicular chain anomalies, contributed to 51.2% of the cases.The stapes and incudostapedial complex are the most commonly involved structures. Conclusion:HRCT provides helpful preoperative clinical information in CMEM.Most cases of aplasia or dysplasia of the ossicular chain, e.g., aplasia/dysplasia of the long process of the incus, aplasia of the stapes'superstructure, and atresia of the oval window were easily identified in preoperative HRCT. However, fixation of the ossicular chain can be elusive in HRCT, and exploratory tympanotomy is needed for a definitive diagnosis.

Modified classification of the isolated congenital middle ear malformations

Modified classification of the isolated congenital middle ear malformations

Congenital Middle Ear Malformation with Common Deafness Gene Mutation Analysis: A Review of 813 Profound Sensorineural Hearing Loss Child Patients.

Deafness gene variants play a key role in inner ear malformations. However, the relationship between congenital middle ear malformations and common deafness genes (GJB2, SLC26A4, and mtDNA) in profound sensorineural hearing loss (SNHL) child patients remains poorly investigated. Here we showed that there was no statistical significance in the total mutation frequency of the three common deafness genes in the middle ear malformation group (21.2%, 41/193) in comparison with the normal middle ear and inner ear group (21.0%, 116/553) (χ2 = 0.0061, p = 0.940). Moreover, the mutation ratio of GJB2 and SLC26A4 in the middle ear malformation group (18.7%, 36/193; 2.6%, 5/193) was not significantly different from that in the normal middle ear and inner ear group (17.7%, 98/553; 2.4%, 13/553) (χ2 = 0.084, p = 0.772; χ2 = 0.0000, p = 1.000). The mutation ratio of GJB2 235delC and GJB2 79G>A in the middle ear malformation group (8.8%, 17/193; 8.8%, 17/193) was almost the same to that in the normal middle ear and inner ear group (8.6%, 48/553; 6.7%, 37/553) (χ2 = 0.0030, p = 0.957; χ2 = 0.9556, p = 0.328). The high jugular bulb subgroup analysis also showed the same results. Our findings suggested that GJB2, SLC26A4, and mtDNA mutations might not be related to the middle ear malformations in profound SNHL child patients. Anat Rec, 303:594-599, 2020. © 2019 American Association for Anatomy.

Isolated Congenital Middle Ear Malformations: Comparison of Preoperative High-Resolution CT and Surgical Findings

To compare preoperative temporal bone high-resolution computed tomography (HRCT) readings to intraoperative findings during exploratory tympanotomy for suspected cases of isolated congenital middle ear malformations (CMEMs) and summarize the malformations that can and cannot be diagnosed with HRCT. A retrospective study was conducted. All cases were confirmed as isolated CMEMs during surgery. Detailed clinical records were reviewed, with a focus on imaging and surgical findings. One hundred and thirty-two patients and 145 ears were reviewed. Ninety cases (62.1%) could be identified as isolated CMEMs and at least one as middle ear anomaly using preoperative HRCT. Fifty-five cases (37.9%) were reported to be completely normal and the patients underwent exploratory tympanotomy to determine the final diagnosis. Stapes fixation, either alone or associated with other ossicular chain anomalies, contributed to 53.1% of the cases. Most cases of aplasia or dysplasia of the ossicular chain, for example, aplasia/dysplasia of the long process of the incus, aplasia of the stapes' superstructure, and atresia of the oval window were easily identified in preoperative HRCT. However, fixation of the ossicular chain can be elusive in HRCT, and exploratory tympanotomy is needed for a definitive diagnosis. HRCT provides helpful preoperative clinical information in CMEM and may obviate the need for middle ear exploration in some cases. The negative findings (anomalies that are difficult to identify through preoperative HRCT) and the positive findings (anomalies that are relatively easy to identify through preoperative HRCT) were summarized.

Congenital middle ear malformation: clinical analysis and discussion of classification

Objective: To analyze the clinical characteristics and appropriate surgical procedures, and discuss the classification of congenital middle ear malformation. Methods: All cases were from the Center of Otorhinolaryngology, the Sixth Medical Center of Department of PLA General Hospital. All of these cases, including 26 male patients (ears) , 10 female patients (11 ears) , aged from 7 to 57 years old, had normal external auditory canal, tympanic membrane, conductive hearing loss, type A tympanogram and negative Gelle's test. Tympanoplasty was performed in all cases. The deformity was classified to three types,i.e., Type I (stapes foot plate mobility): Ⅰa, ossicular chain deformity with normal stapes suprastructure; Ⅰb, ossicular chain deformity with abnormal stapes suprastructure; Type Ⅱ (stapes foot plate fixation): Ⅱ a,normal ossicular chain, Ⅱ b, ossicular chain malformation; and Type Ⅲ: vestibular window osseous atresia or undeveloped, or with round window atresia. The malformation of type Ⅱ and Ⅲ may be accompanied with abnormal facial nerve. In addition, the papers on middle ear malformation published from 1982 to 2017 were analyzed retrospectively. The clinical data of 451 ears malformation were summarized. Results: According to the revisional classification criteria in 37 ear samples from our hospital, 20 ears were type I. 6 type Ⅰa cases were used PORP (partial ossicular replacement prosthesis) to reconstruct the ossicular chain; 14 type Ⅰb cases were used TORP (total ossicular replacement prosthesis) to reconstruct the ossicular chain. For the 5 ears of type Ⅱ, 2 of which were type Ⅱ a and 3 were type Ⅱ b. 4 ear samples of type Ⅱ were implanted with Piston ossicular prosthesis, 1 was implanted with TORP in which the ossificated foot plate was removed with periosteum preserved. 12 ear samples were type Ⅲ, with vestibular window osseous atresia, facial nerve malformation, and stapes suprastructure malformation. The pistons ossicular prosthesis were implanted in vestibular window in 3 ears with facial nerve covering vestibular window partially. The surgery had to be given up in 5 ears, and TORP was implanted in 4 ears at the opening with preserved periosteum at the beginning of the tympanic scala because of facial nerve covering vestibular window totally. 30 ears with complete follow-up data had no sensorineural hearing loss and the average air-bone conduction decreased 23.3±10.7 dB (P<0.05).There were 234 ears of type Ⅰ in 451 ears of congenital middle ear malformation reported in the literature. 113 of which were type Ⅰa, the basic surgery was ossicular chain shaking and artificial or autogenous PORP implantation. Type Ⅰb was 121 ears, with autogenous or artificial TORP and PORP. Type Ⅱ was125 ears, including type Ⅱa 22 ears, Ⅱb 60 ears, and no subclassification for 43 ears. The surgery of type Ⅱ was the same as otosclerosis. The vestibular window atresia of type Ⅲ was 92 ears, the surgery of 17 ears had to be abandoned, the other ears underwent vestibular window, promontory or semicircular canal opening to reconstruct hearing with Piston, autogenous or artificial TORP. Conclusion: Referring to the classification of congenital middle ear malformation combining with appropriate surgical materials and methods, otologists can better understand and choose appropriate surgical method to the middle ear malformation.

Transcanal stapedotomy with 4K ultra-high definition endoscope for congenital malformations of middle ear

Transcanal stapedotomy with 4K ultra-high definition endoscope for congenital malformations of middle ear

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

ObjectivesFacial nerve aberration is the most troublesome situation in congenital malformations of middle ear. The aim of our study is to investigate its imaging and clinical features as well as relevant choice of surgical techniques for hearing improvement. MethodsA retrospective study involving review of clinical data of 227 patients (256 ears) with congenital middle ear anomaly was undertaken, including preoperative computed tomography (CT) data, surgical records and videos. ResultsAberration involving intratemporal facial nerve was found in 82/256 ears (32.03%) with congenital middle ear anomaly. The most common forms of aberration included overhanging over the oval window (50/82 ears, 60.98%), bifurcation (3/82 ears, 3.66%) and transverse over the promontory (3/82 ears, 3.66%), counting for 68.29% (56/82) of the cases with facial nerve aberration. Concomitant stapes malformation was found in 76/82 ears (92.68%) and atresia or stenosis of the oval window in 27/82 ears (32.93%). In 9/82 ears (10.98%) both stapes and oval window was absent. Elective surgeries for the purpose of hearing improvement included stapodotomy + piston implantation, labyrinthotomy, labyrinthotomy + total ossicular replacement prosthesis (TORP) implantation and Vibrant Soundbridge (VSB) implantation. ConclusionThe majority of facial nerve aberration in congenital malformation of middle ear involves displacement of facial nerve, in addition to concomitant malformations of the stapes and/or oval window, which may influence the choice of surgery for hearing improvement. VSB implantation may be considered as a useful option.

Unilateral congenital malformations of middle ear with intact external ear: a review of 64 cases

To describe the operative findings and surgical results of unilateral congenital middle ear malformations with intact external ear. A retrospective review was performed on 64 patients with unilateral congenital middle ear malformations and intact external ear who underwent exploratory tympanotomy from 2011 to 2016. Demographic data, clinical data, high-resolution computed tomography findings, audiometric data and intraoperative findings were collected. Audiological evaluations before and 6months after surgery were analyzed in 47 patients. The most common malformation were mobile stapes with missing incus long process and stapes suprastructure. The air conduction pure tone average was 58.9 ± 10.5dB HL (range 34.4-78.1dB HL) preoperatively and 28.8 ± 10.6dB HL (range 9.4-55.6dB HL) postoperatively (P = 0.000). Twenty-five cases (53.2%) acquired an air conduction hearing gain exceeding 30dB. Mean air-bone gap (ABG) was 44.5 ± 9.4dB (range 22.5-66.4dB HL) before surgery and 15.6 ± 9.3dB (range 0-35.6dB) after surgery (P = 0.000) for an average gain of 28.8 ± 11.5dB. Thirty-four cases (72.3%) showed a postoperative ABG of less than 20dB, 15 had an ABG within 10dB, and 4 had 0dB ABG after operation. No significant difference was observed for air conduction hearing gain regarding age (P = 0.261) or types of malformations (mobile stapes footplate with or without a suprastructure anomaly, P = 0.058). Unilateral congenital middle ear malformations with intact external ear can be complex and diverse. Functional ossiculoplasty for patients with unilateral congenital middle ear malformations can achieve good hearing outcomes.

Reporting and Description for Congenital Middle Ear Malformations to Facilitate Surgical Management

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.

Hearing Rehabilitation in Congenital Middle Ear Malformation.

Microtia and atresia cause significant conductive hearing loss of up to 60 dB HL. The bilateral cases suffer from severely restricted communication abilities and require immediate acoustic stimulation. There is also growing evidence that unilateral cases benefit from an early and selective stimulation of the affected side. Hearing restoration can be performed in selected cases of minor malformation by classic middle ear reconstruction. However, the majority of patients presumably benefit better from a hearing aid. There are 3 main types: active middle ear implants, active bone conduction implants and passive bone conduction implants. All implants improve speech perception, speech recognition, the signal-to-noise ratio and directional hearing. The extent varies among implants and requires further studies. Decision making on the implant type depends on the extent of malformation and hence the preoperative imaging. New scoring systems provide reliable risk stratification. Second it depends on the age of the patient. The active middle ear implants provide a selective stimulation of the affected side and are beneficial if implanted in the first years of life during the maturation period of the auditory system. In conclusion, hearing rehabilitation of congenital atresia should be performed as early as possible. This includes not only the bilateral but also the unilateral affected patients.

The management of patients with congenital malformations of the external and middle ear

Rehabilitation of the patients suffering from with congenital malformations of external and middle ear is a specific area of medicine requiring special knowledge and skills of the audiologists and ear surgeons. This article highlights the issues of epidemiology, classification and methods for the rehabilitation of patients presenting with this pathology that reflect our own experience and the methodology based on it for all phases of both functional and aesthetic surgical inerventions. Special attention is given to the assessment of the effectiveness of the treatment, as part of the evaluation of the quality of medical services. The results were demonstrated in 56 patients aged from 5 to 17 years. 32 of them underwent reconstructive, hearing improving surgery (meatotympanoplasty), 24 patients were managed by means of the implantation of the bone-anchored hearing systems. The assessment of the effectiveness of the treatment was conducted according to the algorithm developed by the authors, including free field pure tone audiometry and validated questionnaires for the assessment of the quality of life (the Glasgow Children's Benefit Inventory).

The implantation of Bonebridge in bilateral congenital malformation of external and middle ear

Objective: To evaluate the auditory efficacy of Bonebridge implantation in patients with bilateral congenital malformation of external and middle ear. Methods: Eleven cases (6 males and 5 females) had unilateral Bonebridge implantation. The age ranged from 8 to 26 and the average age was 16.9. Seven to ten days after operation, the first fitting was undergone. In acoustic sound field, the average auditory thresholds were respectively measured for unaided ears and Bonebridge implanted ears by pure tone auditory (PTA, 0.25, 0.5, 1, 2 and 4 kHz). For the group over 12-year-old, MSTM was applied to evaluate speech discrimination score (SDS). For the other cases, MLNT was used as the test material. The auditory efficacy post Bonebridge implantation would be analyzed and evaluated by comparing the differences between unaided ears and Bonebridge implanted ears. Results: The bone conduction audibility threshold after Bonebridge implantation was as well as the preoperative. The auditory threshold with Bonebridge aided was improved to 25-35 dB HL, when compared to that of the unaided ears in the sound field. The SDS in the group over 12-year-old was improved about 50%; the efficacy was slightly limited for the other two cases (both less than 12 years old). Statistical analysis showed that there were significant differences between unaided ears and Bonebridge implanted ears in the sound field and SDS(P<0.05). Conclusions: The auditory efficacy of Bonebridge is significant and noticeable in patients with bilateral congenital malformation of external and middle ear. Bonebridge provides a new and effective way for patient with congenital malformation of external and middle ear to reconstruct hearing.

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.