Reabilitação auditiva com sistemas Baha® transcutâneo e percutâneo / Hearing rehabilitation with Baha® transcutaneous and percutaneous systems

RESUMO Objetivo Verificar longitudinalmente a influência dos limiares tonais auditivos obtidos com as próteses auditivas ancoradas no osso transcutâneas e percutâneas na percepção da fala em indivíduos com malformação de orelha externa e/ou média e Otite Média Crônica. Método Estudo observacional, retrospectivo, de seguimento longitudinal de 30 indivíduos usuários unilaterais de sistema Baha® transcutâneo e percutâneo, para coleta de dados secundários dos limiares tonais obtidos por meio da audiometria em campo livre e do limiar de reconhecimento de sentenças no silêncio e no ruído nas condições: sem a prótese; no momento de ativação; no primeiro mês de uso (pós 1); e no terceiro mês (pós 2). Resultados Houve diferença significante entre os limiares tonais obtidos nas frequências de 3 e 4kHz, com melhores resultados para o percutâneo em todos os momentos de avaliação. Para os dois sistemas, observou-se melhor desempenho no reconhecimento de sentenças no silêncio e ruído, com diferença significante na ativação (p<0,001), porém manteve-se estável nos demais momentos de avaliação. O sistema percutâneo mostrou melhor benefício no reconhecimento de sentenças no ruído apenas na ativação (p=0,036), quando comparado ao transcutâneo. Conclusão O sistema percutâneo possibilitou melhor audibilidade para as frequências altas; contudo, tal audibilidade não influenciou no reconhecimento de sentenças na situação de silêncio para ambos os sistemas. Para a situação de ruído, melhores respostas foram observadas no sistema percutâneo; porém, a diferença não se manteve no decorrer do tempo.

ABSTRACT Purpose Longitudinally verify the influence of auditory tonal thresholds obtained with transcutaneous and percutaneous bone-anchored hearing aids on speech perception in individuals with external and/or middle ear malformation and chronic otitis media. Methods Observational, retrospective, longitudinal follow-up study of 30 unilateral users of the transcutaneous and percutaneous Baha® system for the collection of secondary data on pure tone thresholds obtained through free field audiometry and sentence recognition threshold in silence and noise in conditions: without the prosthesis; at the time of activation; in the first month of use (post 1); and in the third month (post 2). Results There was a significant difference between pure tone thresholds obtained at frequencies of 3 and 4kHz with better results for the percutaneous technique at all evaluation moments. For both systems, better performance was observed in sentence recognition in silence and in noise, with a significant difference in activation (p<0.001), but it remained stable during the other evaluation moments. The percutaneous system showed better benefit in recognizing sentences in noise only on activation (p=0.036), when compared to the transcutaneous system. Conclusion The percutaneous system provided better audibility for high frequencies; however, such audibility did not influence sentence recognition in the silent situation for both systems. For the noise situation, better responses were observed in the percutaneous system, however, the difference was not maintained over time.

The clinical and imaging presentations of the stapical footplate hernia related to inner ear malformation / 中华放射学杂志

Objective:To summarize the clinical and imaging presentations of stapical footplate hernia related to inner ear malformation (SFH-Re-IEM).Methods:The clinical and imaging data of 19 patients (24 ears) with SFH-Re-IEM from Shandong Provincial ENT Hospital between June 2014 to June 2022 were retrospectively analyzed. The clinical presentations and headache and the high resolution CT (HRCT) and MRI findings including associated inner ear malformation (IEM) type, internal auditory canal (IAC) malformation, bony defect of the stapical footplate, the extent, margin, density and signal of the herniation cysts, the density and signal of ipsilateral intratympanic, cochlear osseous labyrinthitis were recorded.Results:Among 19 SFH-Re-IEM patients, including 14 males and 5 females, 3 (1, 12) years old. All of the affected ears presented sensorineural or mixed hearing loss, 1 ear for moderate, 7 ears for severe and 16 ears for extremely severe. Besides this, the vertigo in 1 case, otalgia and ear fullness in 2 cases were found, and the others were detected accidentally. Among 24 ears with SFH-Re-IEM, 9 ears (37.5%) consisting with incomplete partition type Ⅰ, 3 ears (12.5%) with common cavity, 7 ears (29.2%) with cochlear aplasia, 3 ears (12.5%) with cochlear dysplasia type Ⅱ, and 2 ears (8.3%) with Mondini deformity were found respectively. Four ears (16.7%) were associated with IAC enlargement and the bony defect of IAC fundus, 19 ears (79.2%) were accompanied with dysplasia in the IAC fundus. In all the 24 ears with SFH-Re-IEM, the focal bony defect of the affected stapical footplate and the hemispherical soft-tissue-density herniating cysts protruding into the tympanium were presented on HRCT, and the herniating cysts presenting the cerebrospinal fluid-like signal with the well-defined margin and the ipsilateral normal tympanium were shown on the MRI hydrographic sequence. Two ears underwent the perilymphaticum gadolinium based on MRI, which demonstrated the hypersignal gadolinium in the perilymphatic space entered into the herniating cysts. Two ears were accompanied with the cochlear osseous labyrinthitis.Conclusion:The ears with IEM-Re-SFH usually present the serious hearing loss. The ipsilateral severe IEM, focal bony defect of the stapical footplate and perilymph herniating are characteristic imaging appearances.

Sordera neurosensorial congénita con malformación de oído interno ligada al X en una familia mexicana / Congenital sensorineural deafness X-linked, with inner ear malformation in a Mexican family

Introducción. Las sorderas o hipoacusias prelinguales son de etiología genética entre el 60 y el 68% de los casos; de estos, del 20 al 40% son malformaciones del oído interno. De los casos de hipoacusia no sindrómica ligada al X se han descrito siete tipos. De las malformaciones de oído interno, la partición coclear incompleta tipo III es la menos frecuente.Objetivo. Presentar el reporte clínico-genético de una familia mexicana, con indi-viduos varones afectados por sordera neurosensorial congénita con malformación de oído interno. Material y Métodos. Se realizó estudio de una familia en la que nueve miembros presentaban sordera. Se estudiaron cuatro de ellos y una madre sin manifestaciones, a través del estudio clínico general por médico genetista, el estudio audiológico (otos-copía y audiometría) por médico audiólogo y el estudio de tomografía computada (TC) por médico radiólogo.Resultados. Los pacientes estudiados presentaron sordera neurosensorial congéni-ta, de severa a profunda bilateral. A través de la TC, se evidenció malformación de oído interno. Tres pacientes presentaron partición coclear incompleta tipo III y un paciente partición incompleta tipo I. Debido al estudio clínico y al árbol genealógico, se definió diagnóstico de hipoacusia neurosensorial no sindrómica ligada al X. La TC de la madre sin manifestaciones no presentó evidencia de malformaciones en oído interno (MOI).Conclusión. El estudio de imagen es fundamental para definir presencia o no de MOI en todos los pacientes con hipoacusia y así poder guiar la terapéutica y el aseso-ramiento genético, así como realizar los estudios moleculares más adecuados

Introduction. The pre-lingual deafness or hearing loss are of genetic cause in be-tween 60% and 68% of cases, among these, between 20% and 40% are malforma-tion of the inner ear. From the non-syndromic hearing loss cases that are linked to the X chromosome, seven types have been described. Among these inner ear malforma-tions, incomplete cochlear partition type III is the less frequent.Objective. Present the clinical genetical report of a Mexican family, with male in-dividuals affected by congenital neurosensory deafness with inner ear malformation.Materials and methodology. A study on a family in which nine members were affected by deafness was done. Four of them, plus a mother without manifestation, were studied through a general clinical study by a geneticist, an audiological study (otoscopy and audiometry) by an audiologist, and a computed tomography (CT) scan by a radiologist.Results. The studied patients presented congenital neurosensory deafness, from se-vere to deep bilateral. Via the CT, the inner ear malformation was made clear. Three of the patients presented incomplete cochlear partition type III and one patient in-complete cochlear partition type I. Due to the clinical study and the family tree, it was diagnosed non-syndromic neurosensory deafness linked to X. The CT of the mother without manifestation did not show evidence of inner ear malformations.Conclusion. The study by image is fundamental to define whether there is or not a presence of inner ear malformations in any patient with heading loss to be able to guide the therapeutics and the genetic counseling, as well as to make more accurate molecular studies

Frequency of Inner Ear Anomalies Among Cochlear Implant Candidates: A Case Study in the Northwest of Iran

@#Introduction: Globally hearing impairment occurs in about 1 to 2 per 1000 live births. The etiology of severe sensorineural hearing loos (SNHL) is complex and multifactorial. Congenital inner ear abnormality is a major cause of hearing loss in children and cochlear implantation (CI) is a proper treatment in these children. Preoperative CT scan of the temporal bones are used to evaluate inner ear malformations in CI candidates. The frequency of internal ear malformations reported on temporal scan could varied from 20% to 30%. The aim of this study was to evaluate the frequency of inner ear malformations in CT scan of cochlear implant candidates . Methods: In a retrospective cross-sectional descriptive study, 201 infants (105 boys and 96 girls ) with the mean age of 20.5 ± 14.01 month with severe SNHL who are CI candidates were examined by temporal bone imaging with multi-slice CT from March 2014 to March 2015 in CI center of Tabriz University. Results : CT revealed abnormalities of the inner ear in 26 (13%) of infants that most of them with 10 (38.5%) was enlarged vestibular aqueduct (EVA). Also, 31 infants (15.4%) had abnormalities in outer, middle ear or in mastoid aeration. Conclusion: Temporal bone imaging with CT is an essential method and could be suggested as a proper first step for evaluating inner ear malformations in CI candidates.

Auricular cartilage multi-point suspension fixed to craniofacial deep fascia in correcting mild to moderate cupped ear malformation / 中国修复重建外科杂志

Objective: To explore the feasibility and effectiveness of using auricular cartilage multi-point suspension fixed on deep craniofacial fascia in correcting mild to moderate cupped ear malformation. Methods: Between January 2014 and March 2016, 22 patients (12 males and 10 females) with mild to moderate cupped ear malformation were admitted, aged from 6 to 28 years, with an average age of 15 years. Sixteen cases were unilateral and 6 cases were bilateral. According to Tanzer classification, there were 18 sides of type Ⅰ and 10 sides of type Ⅱ. The otocranial groove incision was selected to expose and release the posterior auricular muscles and ligaments. The abnormal structure of auricle subunits was remolded. The auricle cartilage was suspended and fixed on the deep craniofacial fascia with non absorbable line to remodel the shape and position of auricle. Results: The incision healed by first intention, without hematoma, infection, and skin necrosis. All the patients were followed up 3-48 months, with an average of 12 months. In addition to 1 case of slippage of the fixed line knot, the effect was good after being suspended and fixed again, the auricles of the other patients were not drooping and tilted forward, the shape of the outer ear was good, the ear boat was obvious, the shape of the upper and lower feet of the ear wheel and the pair of ears was natural, the bilateral symmetry was good, and the patients and their families were satisfied. Conclusion: Auricular cartilage multi-point suspension fixed on deep craniofacial fascia is effective in the treatment of mild to moderate cupped ear malformation.

Numerical Analysis on Sound Compensation Performance of Round Window Stimulation in Middle Ear Malformation / 医用生物力学

Objective To study the influence of middle ear malformation on the performance of round window stimulation, so as to provide references for optimization of the round window stimulation middle ear implant. Methods The finite element model of human ear including an asymmetrical two-cavity non-helical cochlea was constructed and compared with experimental data to verify reliability of the model. Based on this model, the effects of three kinds of middle ear malformation, i.e., ossicular chain fixation, ossicular chain fusion and ossicular defect on round window stimulation were simulated by changing material properties of the corresponding tissues. Results The middle ear malformation mainly affected the low-frequency performance of round window stimulation. The ossicular chain fixation and the ossicular chain fusion had a deteriorating effect on round window stimulation. The stapes fixation had the greatest effect on the performance of round window stimulation, with the reduction as high as 47.93 dB. Ossicles defects could improve the performance of round window stimulation, with the maximum increment of 6.24 dB. Conclusions The middle ear malformation had an effect on the low frequency performance of round window stimulation. It is necessary to specifically increase output of the actuator when implanting the round window stimulation middle ear implant.

Malformaciones del oído externo y medio en pediatría: características clínico audiológicas y tratamiento / External and middle ear malformations in pediatrics: Clinical and audiological features and treatment

Objetivo: Describir las características clínicas y audiológicas de los pacientes que presentan malformaciones del oído externo y medio. Detallar la intervención con procesadores de conducción ósea y los distintos implantes como tratamiento para niños con hipoacusia auditiva moderada conductiva o mixta debido a este tipo de anomalías congénitas. Material y métodos: Se realizó un estudio prospectivo, longitudinal y observacional de 223 pacientes consecutivos con malformación auricular bilateral o unilateral atendidos en una primera consulta o en seguimiento desde octubre de 2013 hasta noviembre de 2014. Los datos a analizar de estos pacientes incluyeron: edad, sexo, existencia de otras malformaciones o síndromes asociados, uni o bilateralidad de la malformación, clasificación de la misma en grados, características del conducto auditivo externo, del nervio facial y la existencia de colesteatoma. Se realizaron evaluaciones audiológicas adecuadas para la edad madurativa del paciente. Se describen los distintos tratamientos realizados (procesadores de conducción ósea con vincha o implante). Resultados: 33% (73/223) presentó malformación auricular bilateral, con una mediana de edad de 2 años (rango 1-14 años). El 67%, (150/223)) presentó malformación auricular unilateral: 49/150 en el lado izquierdo; 101/150 en el lado derecho, con una mediana de edad de 7 años, (rango 1-16 años). El 43% (96/223) sexo femenino y el 57% por ciento (127/223) sexo masculino. El 31% (23/73) de los niños con malformación bilateral y el 16% (24/150) de los niños con malformación unilateral presentaban síndromes congénitos. Con respecto al grado de malformación, 56% de los pacientes presentaron malformación auricular grado III/IV, 28% malformación grado II y 16% malformación grado I. En el 68% de los casos presentaban atresia de conducto auditivo externo, 20% estenosis severa y 12% estenosis leve con malformación de oído medio. El 3,5% de los pacientes (5/223) tenía parálisis facial. El 4,5% de los pacientes (8/223) presentó colesteatoma en un conducto auditivo externo con estenosis severa. Se diagnosticó hipoacusia conductiva o mixta moderada en el 98,6% de los oídos con malformación. El 1,4% presentó hipoacusia neurosensorial. Dieciocho niños con malformación bilateral menores de 18 meses de vida fueron equipados con dispositivos de conducción ósea con vincha. En cuatro de estos pacientes los dispositivos provistos fueron bilaterales. En los niños mayores de cinco años se indicó cirugía de implante en 14 casos. En casos unilaterales se indicó procesador de sonido con vincha en seis niños menores de 18 meses de vida. Cinco de ellos ya han sido proporcionados y la están utilizando. En niños mayores de cinco años se indicó cirugía con implantes osteointegrados en 6 casos (AU)

Objective: To describe the clinical and audiological features of patients presenting with external and middle ear malformations. To detail the intervention with bone conduction processors and different implants in the treatment of children with moderate conductive or mixed hearing loss, conductive or mixed due to this type of congenital anomalies. Material and methods: A longitudinal, prospective, observational study was conducted in 223 consecutive patients with bilateral or unilateral aural malformations seen at the first visit or in follow-up between October 2013 and November 2014. The following data were analyzed: age, sex, associated malformations or syndromes, uni- or bilaterality of the malformation, classification of the malformation, characteristics of the external ear canal, the facial nerve, and presence of cholesteatoma. Adequate audiological evaluations for age were performed. The different management options are described (bone conduction devices with headband or implant). Results: 33% (73/223) presented with a bilateral ear malformation at a median age of 2 years (range, 1-14 years); 67% (150/223)) had a unilateral ear malformation: 49/150 on the left side and 101/150 on the right side, at a median age of 7 years (range, 1-16 years). Overall, 43% (96/223) were female and 57% (127/223) were male. Of all the children, 31% (23/73) with a bilateral malformation and 16% (24/150) with unilateral malformation had congenital syndromes. Regarding the degree of malformation, 56% of the patients had a grade III/IV ear malformation, 28% a grade II malformation, and 16% a grade I malformation; 68% of the cases had external ear canal atresia, 20% severe stenosis, and 12% mild stenosis with a middle ear malformation; 3.5% of patients (5/223) had facial paralysis; 4.5% (8/223) had cholesteatoma in the external ear canal with severe stenosis. Moderate conductive or mixed hearing loss was diagnosed in 98.6% of the ears with malformation. Sensorineural hearing loss was observed in 1.4%. Eighteen children with bilateral malformation under 18 months of age were fitted with a bone conduction device with a headband. Four of these patients needed bilateral devices. In 14 children over five years of age, implant surgery was indicated. In six children under 18 months of age with a unilateral malformation, a sound processor with headband was indicated. Five of them have already been fitted with the device. mplantation of a bone-anchored hearing aid was indicated in six children over five years of age (AU)

ЭХЭМҮТ-Н ХЗП-Н СОНСГОЛ ЗАСЛЫН КАБИНЕТААР ҮЙЛЧЛҮҮЛСЭН ГАДНА ЧИХНИЙ ГАЖ ХӨГЖИЛТЭЙ ХҮҮХДҮҮДИЙН ХЯНАЛТЫН СУДАЛГААНЫ ЗАРИМ ҮР ДҮН / Шинэ санаа Шинэ нээлт

@#BACKGROUND: Microtia is often associated with hearing loss and patients typically require treatment for hearing impairment and surgical ear reconstruction. The occurrence of microtia is of public health importance in part due to the psychosocial sequelae, including the stigma associated with malformations of the ear and the burden of undergoing multiple surgeries In addition, greater than 90% of individuals with microtia experience conductive hearing loss on the affected side. Although children with microtia-anotia are at a greater risk of delayed language development and attention deficit disorders. METHODS: Our study has a 173 childrens from the UB city and countryside. 23 children has a bilateral microtia and anotia. 6 patient wearing BAHA soft band from 6 months age. 150 patients have an unilateral microtia. Microtia was found more commonly in males, unilateral and right sided. Even children with bilateral microtia may have wearing BAHA soft band before 6 months age. RESULTS: External ear malformations are more commonly found in males. Sex ratio 2:1. From other studies right ear malformations are more common in male children. In our study children who have bilateral ear abnormalities wearing BAHA softband and language therapy from 6 months age to able to have normal language development. Children who had surgical treatment and using hearing aid can have normal social life and increased quality of life. CONCLUSION: Early screening in children who have external ear malformation, monitoring language development, wearing BAHA softband from 2 months age who have bilateral ear malformation and one ear affected other one is normal children wear BAHA softband increases children speech and language development.

The Inner Ear Malformation Diagnosed by High-resolution Computed Tomography for its Classification in Sensorineural Hearing Loss / 听力学及言语疾病杂志

Objective To study sensorineural hearing loss classification and incidence of inner ear malformations based on the image of high-resolution computed tomography.Methods The investigation took the form of a retrospective review of CT findings relating to the 5 398 ears of outpatients in the general PLA hospital in the last 10 years.The inner ear malformations diagnosed by CT were classified according to the methods proposed by Sennaroglu's 2010.Results A total of 1 640 ears of inner ear malformations were found in 5 398 ears with sensorineural hearing loss by CT examination.The incidence of the inner ear malformation was 30.38% (1 640/5 398).The epidemiological information of 1 660 ears of inner ear malformation according to Sennaroglu's classification was as follows: cochlea information was 53.66%(880/1 640)and non-cochlea information was 46.34%(760/1 640)of the group.Eight hundred and eighty ears of cochlea malformation consisted of these types: 5 ear of Michel deformity, 23 cochlea of aplasia, 6 common cavity deformity, 69 cochlea of hypoplasia with 19 CH-I , 16 CH-II, and 34 CH-III, 777 cochlea incomplete partition with 44 IP-I , 703 IP-II and 30 IP-III.Seven hundred and sixty ears of non-cochlea malformation consisted of 680 large vestibular aqueducts, 80 simple vestibular/ semicircular canal/ internal auditory canal malformations.IP-II type and large vestibular aqueduct were related to the vestibular aqueduct malformation, making up 84.33%(1 383/1 640)ears of the whole malformation group.Conclusion The results suggest that 30.38% inner ear malformation can be found in ears with sensorineural hearing loss,higher than reported based on the image of high-resolution computed tomography.Sennaroglu's 2010 classifications have instructive significance in investigating the status of inner ear malformations.

Analysis of clinical characteristic of the middle ear malformation with cholesteatoma / 中华显微外科杂志

Objective To discuss the clinical characteristic of middle ear malformation with cholesteatoma.Methods From September,2011 to November,2016,23 cases of middle ear malformation with cholesteatoma were collected.Their symptoms,ENT examination,temporal bone high resolution computed tomography (HRCT) and intraoperative findings were analyzed.Results All 23 patients had hearing loss.90.0％ patients had ear discharge and tinnitus.86.96％ patients had perforation in pars flaccida of tympanic membrane.95.7％ patients had poor mastoid gasification.73.9％ patients had undeveloped or dysplastic tympanic antrum.100％ patients had destruction of the ossicular chain.56.5％ patients had malformation of the ossicular chain.52.2％ patients had exposed facial nerve.30.4％ patients had hyperplasia of the attic bone.Conclusion The symptoms and physical examinations of middle ear malformation with cholesteatoma are the same as middle ear cholesteatoma.Poor mastoid gasification can usually be detected on temporal bone HRCT.The most common malformation is dysplastic tympanic antrum,followed by malformation of the ossicular chain,exposed facial nerve and hyperplasia of the attic bone.Temporal bone HRCT can indicate the malformation and destruction quite well.

Mutations in SLC26A4 Gene and Relevant Phenotype Analysis in Fifty-nine Cases of Enlargement of Vestibular Aqueduct(EVA)Syndrome Children in Guangdong District / 听力学及言语疾病杂志

Objective The molecular etiology of hearing impairment in Guangdong District has not been thor-oughly investigated.SCL26A4 gene mutation and relevant phenotype were analyzed in this study.Methods The coding exons of SLC26A4 were analyzed in 59 EVA cases.Those SLC26A4 gene mutations patients were examined by temporal bone CT.Results Fifty-nine cases were SLC26A4 mutations deafness patients,and 21 cases (35. 59%)and 38 cases (64.41%)patients with SLC26A4 biallelic allele (compound homozygous or heterozygous)and monoallelic gene mutation,including 16 cases of SLC26A4 gene IVS7-2 A> G homozygous mutations,2 cases of 2168A>G homozygous mutations and 3 cases of IVS7-2A>G,2168 A > G compound heterozygous mutations in children with CT showing bilateral enlarged vestibular aqueduct or other types of inner ear malformations.Thirty-one patients were IVS7-2A>G heterozygous for SLC26A4 mutation and seven 2168 A > G heterozygous muta-tion.Four patients with SLC26A4 gene mutations were confirmed to have enlarged vestibular aqueduct with Mondini dysplasia.Two patients with normal phenotype ,and others were enlarged vestibular aqueduct.Conclusion Muta-tions in the SLC26A4 gene with enlarged vestibular aqueduct patients were frequently found in Guangdong District.IVS7-2A>G mutations rate were highest,followed by 2168 A > G.We established the new strategy that detects SLC26A4 mutations prior to the temporal bone CT scan to find enlarged vestibular aqueduct and inner ear malforma-tion patients .

Microtia: definición, clasificación y tratamiento: revisión de la literatura / Microtia: definition, classification and treatment: Literature review

El pabellón auricular es una importante unidad estética en el ser humano. Existe un grado variable de malformaciones, en Chile tiene una incidencia de 1 por cada 5600 nacidos vivos. Anatómicamente el pabellón auricular está compuesto por tres estructuras principales: complejo hélix antihélix, concha y lóbulo. Es según la anomalía que se presente en la anatomía externa la forma en que se clasificará, existiendo diversos sistemas para esto, algunos de ellos con utilidad anátomo quirúrgica.El eje principal del tratamiento de estas anomalías es la reconstrucción quirúrgica, teniendo disponible en la literatura variadas técnicas, como son el uso de implantes, reconstrucción protésica y la reconstrucción con injerto autólogo de cartílago costal, entre otras. Reinisch ha sido pionero en el uso de implantes para la reconstrucción, mientras que diversos autores han mostrado resultados satisfactorios con el uso de prótesis osteointegradas. Sin embargo, la técnica más aceptada y utilizada es la reconstrucción con injerto autólogo de cartílago costal, la cual fue descrita en sus inicios por Tanzer, Walton y Beahm, siendo perfeccionada posteriormente por Brent, quien propuso las bases para su desarrollo moderno. En la actualidad los métodos quirúrgicos más aceptados y utilizados son los descritos por Nagata y Firmin, ambos con excelentes resultados.

The ear is an important aesthetic unity in humans. There is a varying degree of malformations, Chile has an incidence of 1 per 5600 live births. Anatomically the pinna is composed of three main structures: helix antihelix complex, shell and lobe. It is according to the anomaly present in the external anatomy how it is classified, various systems exist for this, some of them with anatomic surgical utility. The main focus of treatment of these anomalies is the surgical reconstruction, taking various techniques available in the literature, such as the use of implants, prosthetic reconstruction and reconstruction with autologous rib cartilage graft, among others. Reinisch has pioneered the use of implants for reconstruction, while several authors have shown satisfactory results with the use of bone anchored prosthesis. However, the most accepted and used technique is the reconstruction with autologous rib cartilage graft, which was described at the beginning by Tanzer, Walton and Beahm and was later perfected by Brent, who proposed the foundation for modern development. Currently the most accepted and used surgical methods are described by Nagata and Firmin, both with excellent results.

CT and MRI diagnostic value of the congenital inner ear malformation incomplete partition type Ⅱdeformity combined with spontaneous CSF otorrhea / 实用放射学杂志

Objective To discuss the diagnostic value of high resolution computed tomography (HRCT)and MRI in the congenital inner ear malformation incomplete partition type Ⅱ deformity(Mondini deformity)combined with spontaneous cerebrospinal fluid (CSF)otorrhea.Methods The imaging data including the multiplanar reformation(MPR)images of HRCT,curved planar reforma-tion(CPR)images and MR hydrography images in three patients complicated with recurrent meningitis were retrospectively ana-lyzed.The anatomic changes of the inner ear and middle ear on the affected side were observed,the clinical and imaging features of this disease were summarized.Results One patient had bilateral incidence of the incompletely separated inner ear,combined with semicircular canal dysplasia and cochlear pipe dilation.Two patients had unilateral onset,one of them had concurrent facial nerve ca-nal dysplasia.Humble stapes floor and the defect of the bottom of the internal auditory canal were seen in all the three patients.Con-clusion HRCT with post-processing technology,and MRI have great significance in the diagnosis of the congenital inner ear malfor-mation incomplete partition type Ⅱ deformity (Mondini deformity)combined with spontaneous CSF otorrhea.

Three Cases of Bilateral Semicircular Canal Aplasia with Normal Cochlear Development in the Same Family Member / 대한이비인후과학회지

Semicircular canal aplasia is rare congenital inner ear anomaly. During embryogenesis, congenital malformation of vestibular labyrinth usually associates with cochlear anomalies. Two cases of semicircular canal aplasia with normal or near-normal cochlear development was reported in the English literature. We present three patients with computed tomographic findings of bilateral total semicircular canal aplasia with normal cochlear development in the same family member. Two patients had significant conductive hearing loss due to congenital stapedial anomalies and the other was congenital deafmute. Temporal bone CT scan revealed total absence of the semicircular canals bilaterally and both cochleas were normal in three cases.