Objective:To explore the value of high resolution computed tomography（HRCT） combined with Magnetic Resonance Imaging（MRI） in the diagnosis of inner ear malformation. Methods:HRCT and MRI data of 82 patients with inner ear malformations were analyzed retrospectively. HRCT MPR and CPR reconstruction of the inner ear structure, facial nerve canal and oblique sagittal MRI reconstruction of the internal auditory canal were performed. The inner ear malformations were classified, the conditions of facial nerve canal and cochlear nerve were evaluated. The association between inner ear malformation and cochlear nerve dysplasia were analyzed by Chi-square test with continuity correction. Results:Among the 82 patients with inner ear malformations,there were 49 cases of bilateral symmetry, 11 cases of bilateral asymmetry and 22 cases of unilateral inner ear malformations. Respectively, the most prevalent types were IP-Ⅱ（42.96%）, dilatation of atrium aqueduct（18.31%） and malformations of atrium and semicircular canal 19.72%. Out of 50 cases of cochlear malformations,only 3 were isolated cochlear malformations, and the rest were accompanied by other malformations of varying degrees. In the 67 ears examined by MRI, 26（38.81%） had cochlear nerve deficiency（CND）, and the incidence of CND varied with different types of inner ear malformations. Out of 142 ears, 28（19.72%） had abnormalities of the facial nerve canal. Conclusion:HRCT combined with MRI can accurately distinguish the types of inner ear malformation and effectively evaluate the facial nerve canal and cochlear nerve, and further provides the important finger and Guide value for the clinician to formulate the reasonable treatment and the operation plan.
Humans ; Ear, Inner/diagnostic imaging* ; Magnetic Resonance Imaging/methods* ; Retrospective Studies ; Female ; Male ; Tomography, X-Ray Computed/methods* ; Child ; Adolescent ; Adult ; Child, Preschool ; Cochlear Nerve/diagnostic imaging* ; Facial Nerve/abnormalities* ; Cochlea/abnormalities* ; Infant ; Young Adult

OBJECTIVE: To analyze the abnormal conditions of the affected ear and the contralateral ear of patients with unilateral Meniere's disease and the prevalence of bilateral abnormalities among these unilateral Meniere disease population, providing reference for the clinical treatment strategies for Meniére disease. METHOD: A retrospective analysis of 106 Meni6re disease cases was performed, the abnormal incidence of the affected ears, the contralateral ears and the bilateral abnormalities were calculated, and the disease characteristics were analyzed. RESULT: The bilateral ears abnormal incidence of unilateral Meniére disease was 35. 85% (38/106); the cochlear symptoms of the contralateral ears often occurred 2. 25 years later of the symptoms of Meni6re disease; contralateral cochlear symptoms included at least two symptoms of tinnitus, deafness and ear fullness; 39. 47%(15/38) patients with bilateral abnormalities would appear binaural hearing impairment. CONCLUSION This study showed that about one-third of unilateral Meniére diseases have binaural symptoms, among which about one-third would occur bilateral hearing loss. Therefore, it is necessary to consider the course of disease and the symptoms of the contralateral ear before taking damage or destructive method for treating Meniére's disease clinically.
Cochlea ; physiopathology ; Deafness ; Ear ; abnormalities ; Hearing Loss, Bilateral ; epidemiology ; Humans ; Incidence ; Meniere Disease ; epidemiology ; Prevalence ; Retrospective Studies ; Tinnitus

OBJECTIVE: Analyze the data of the patients with sensorineural hearing loss in China and study the classification and incidence of inner ear malformationsby the high-resolution computed tomography. METHOD: The investigation took a retrospective review of CT findings relating to the 2,747 cases of outpatients. The inner ear malformations diagnosed by CT were classified according to the methods proposed by Sennaroglu. RESULT: (1)843 cases of inner ear malformations were found in 2747 cases of patients with sensorineural hearing loss by CT examination. The incidence of inner ear malformation was 30.69%(843/2747). (2) The epidemiological information of 843 cases of inner ear malformation according to Sennaroglu's classification was as follows: cochlea was 52. 31%(441/843), simple vestibular aqueduct was 40.33%(340/843), simple vestibular/ semicircular canal/internal auditory canal were 7. 35%(62/843) of the group. (3) 441 cases of cochlea malformation were consisted of these types of malformation: Michel deformity was 1.13% (5/441), cochlear aplasia was 1. 81% (8/441), common cavity deformity was 3. 17% (14/441), incomplete partition type I was 8. 62% (38/441), cochlea hypoplasia was 9. 07% (40/441) and incomplete partition type II was 76. 19% (336/441) of the group. CONCLUSION The results suggested that 30. 69% cases of inner ear malformation can be found in patients with sensorineural hearing loss, which is more higher than reported by the high-resolution computed tomography. Sennaroglu's classification is instructively significant in investigating the status of inner ear malformations.
China ; Cochlea ; Ear, Inner ; abnormalities ; Hearing Loss, Sensorineural ; etiology ; Humans ; Outpatients ; Retrospective Studies ; Semicircular Canals ; Temporal Bone ; Tomography, X-Ray Computed ; Vestibular Aqueduct ; Vestibule, Labyrinth

OBJECTIVE: Explore the relationship between the pathogenic mutations of SLC26A4 gene and inner ear malformation, and analyze the feasibility of genetic testing to help current diagnosis in part of children with sensorineural hearing loss. METHOD: 2094 cases of children were detected by SLC26A4 with the method of DNA sequence. CT phenotypes of those children were classified according to the method proposed by Sennaroglu. We analyzed the relationship between the pathogenic mutations of gene and the CT phenotypes. RESULT: (1) 685 cases of inner ear malformations were found in 2094 cases of children with sensorineural hearing loss by CT examination (371 cases of cochlea malformation were consisted of the follow types of malformation. Michel deformity was 6 cases, cochlea aplasia was 8 cases, common cavity deformity was 12 cases, incomplete partition type I was 27 cases, cochlea hypoplasia was 30 cases and Mondini malformation was 288 cases); Vestibular aqueduct was 265 cases; Vestibular/semicircular canal/internal auditory canal were 49 cases, normal was 1409 cases. (2) The DNA sequence results revealed that 465 cases carried pathogenic mutations (Bi-allelic mutations) of SLC26A4 gene, among which 135 cases were homozygous, 330 cases were compound heterozygous. (3) Pathogenic mutations of SLC26A4 gene detected 100% (465/465) in the group related to vestibular aqueduct malformation. CONCLUSION The results suggest that pathogenic mutation of SLC26A4 gene is closely related to the CT phenotype of vestibular aqueduct malformation. Detecting of pathogenic mutations for hearing loss is binging the possibility to identify children with inner malformations at an early stage. As a consequence, it will improve the current diagnosis and therapeutical option.
Alleles ; Child ; Cochlea ; Deafness ; Ear, Inner ; abnormalities ; Genetic Testing ; Hearing Loss ; Hearing Loss, Sensorineural ; Humans ; Membrane Transport Proteins ; genetics ; Mutation ; Phenotype ; Semicircular Canals ; Sulfate Transporters ; Temporal Bone ; Vestibular Aqueduct ; Vestibule, Labyrinth

OBJECTIVE: To review the classification of cochlear modiolus deficiency and decision on surgical approach for above case,in order to provide mastery for cochlear implant (CI) indication. METHOD: Basing on temporal bone HRCT pre-operation, CI subjects with modiolus deficiency were defined as following groups: (1) deficiency caused by cochlear dysplasia (Mondini malformation); (2) deficiency caused by dysplasia of cochlear and vestibule (Common cavity malformation); (3) deficiency caused by absence of internal acoustic meatus fundus (IP-III malformation). Three types of surgical approach were utilized: type I, electrode array was introduced through facial recess, enlarged the round window, type II, opened the surface of chchlea, electrode array was introduced through facial recess, fenestration on posterior promontory and then inserted around lateral wall of inner-cochlear cavity. type III, electrode array was introduce through fenestration of lateral semicircular canal and then placed close to the bony wall of common cavity. RESULT: One hundred and sixty-six cochlear modiolus deficiency cases were identified into 3 groups as following: 135 Mondini malformation cases into group a, 18 common cavity malformation cases into group b, and 13 IP-III malformation cases into group c. Surgical approach: type I were used in 136 cases (123 Mondini cases and 13 IP-III cases), while approach type II in 12 cases (12 Mondini cases), and approach type III in 18 cases (18 common cavity cases). Income post-operation of CI: For group a (Mondini malformation), post-activation mean hearing threshold in sound field was 65 dB, speech recognition score is 95% (single finals test) and 25% (signal initials test), while it was 80 dB, 60% and 0 for group b (Conmon cavity malformation), and it was 55 dB, 100% and 45% for group c (IP-III malformation). CONCLUSION The income of speech recognition score for cochlear modiolus deficiency was relatively poor, group b was worst and group c was best, while group a moderate.
Cochlea ; abnormalities ; surgery ; Cochlear Implantation ; methods ; Cochlear Implants ; Ear ; Ear, Inner ; abnormalities ; Female ; Humans ; Male ; Postoperative Period ; Round Window, Ear ; surgery ; Semicircular Canals ; surgery ; Speech Perception ; Temporal Bone ; Vestibule, Labyrinth ; abnormalities

OBJECTIVE: To discuss the surgical approach of cochlear implantation in patients with common cavity. METHOD: Seventeen patients with common cavity underwent cochlear implantations through facial recess approach or transmastoid lateral semicircular canal approach,according to the preoperative imaging and audiological evaluation. RESULT: Common cavity was opened and electrodes were inserted smoothly in all cases. Facial recess approach was used in 3 patients, while transmastoid lateral semicircular canal approach was used in the others. Intraoperative "gush" occurred in 4 cases. None of the cases developed intraoperative or postoperative complications, such as facial paralysis, meningitis and cerebrospinal fluid leakage. All cases had improvements in hearing. Atypical postoperative EABR responses were detected, the average free filed hearing threshold was 65 dB HL, the average speech recognition score of Chinese vowels was 75%, and the average speech recognition score of Chinese consonant was less than 10%. CONCLUSION (1) Selection of surgical approach for patients with common cavity: if the basal turn of cochlear could be distinguished at the posterior tympanum side of common cavity, the facial recess approach was used; if the cochlear,vestibule and the lateral semicircular canal merged to be a spherical cavity, the transmastoid lateral semicircular canal approach was used. (2) Surgical approach of cochlear implantation affected the postoperative outcomes. (3) Outcomes of cochlear implantation in common cavity patients were much poorer than normal cochlear cases.
Adolescent ; Child ; Child, Preschool ; Cochlea ; abnormalities ; Cochlear Implantation ; methods ; Cochlear Implants ; Ear Diseases ; surgery ; Ear, Inner ; abnormalities ; Female ; Humans ; Infant ; Male ; Retrospective Studies ; Semicircular Canals ; abnormalities ; Vestibule, Labyrinth ; abnormalities

OBJECTIVE: To investigate the clinical experiences and access the auditory performance and speech intelligibility of cochlear implantees with inner ear malformations. METHOD: A retrospective study was performed on the surgical characteristics and the mapping characteristics after implantation of patients who received mult-channel cochlear implantation from 2000 to 2007 in Xijing Hospital. Matched with normal implanted, rank -sum test was performed to compare scores of categories of auditory performance (CAP) and speech intelligibility rating(SIR). RESULT: After the regular rehabilitation, the long term follow-up of the auditory performance and speech intelligibility were very positive. There was no significant difference between normal group and malformations group. CONCLUSION The cochlear implantation can be performed safely in patients with malformed inner ear. The results suggest that cochlear implantation could also provide long-term benefits to those malformed inner ear cases.
Child ; Child, Preschool ; Cochlea ; abnormalities ; surgery ; Cochlear Implantation ; rehabilitation ; Cochlear Implants ; Deafness ; rehabilitation ; surgery ; Ear, Inner ; abnormalities ; surgery ; Female ; Humans ; Infant ; Male ; Retrospective Studies ; Speech Perception ; Surveys and Questionnaires ; Treatment Outcome

OBJECTIVE: To discuss the indication and approach of cochlear implantation with common cavity inner ear malformation. METHOD: Through imaging examination preoperatively for profound sensorineural hearing loss patients, we found 6 cases with common cavity inner ear malformation, in which cochlear, vestibule and lateral semicircular canal formed a big cavity. Among these cases, five had residual hearing, in the others, residual hearing was not detected. RESULT: Among these cases with common cavity, 5 cases with residual hearing were treated with cochlear implantation, in 3 cases we selected round window approach, in the other 2 cases, we implanted the electrodes through the joint of the common cavity and posterior semicircular canal. Postoperatively, All these 5 patients have auditory response with cochlear implantation. The patient without residual hearing was not given cochlear implantation. CONCLUSION In patients with common cavity inner malformation, those who have residual hearing have indication for cochlear implantation, but it must be emphasized that their effect may not be as good as patients without inner ear malformation. In those patients without residual hearing, cochlear implantation should better not be performed.
Adolescent ; Child ; Child, Preschool ; Cochlea ; abnormalities ; Cochlear Implantation ; methods ; Contraindications ; Ear, Inner ; abnormalities ; Female ; Humans ; Male ; Semicircular Canals ; abnormalities