[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.

Conductive hearing loss with a "dry middle ear cleft"-A comprehensive pictorial review with CT.

Conductive hearing loss (CHL) commonly results from middle ear fluid and inflammation (otitis media). Less commonly in patients with CHL, the middle ear cleft is well aerated or 'dry' with absence of soft tissue or fluid clinically and on imaging. There are numerous causes for this but they can be clinically challenging to diagnose. This pictorial review aims to illustrate and discuss the CT features of both common and less common causes of CHL in patients with a "dry middle ear cavity".

Influence of infancy care strategy on hearing in children and adolescents: A longitudinal study of children with unilateral lip and /or cleft palate.

OBJECTIVES: To evaluate the relation between ventilation tube insertion, otitis media with effusion duration and otologic outcomes in unilateral cleft lip and/or cleft palate children from infancy to teenage age. DESIGN AND POPULATION: Retrospective longitudinal charts review of patients from the multidisciplinary cleft team of the University Hospital of Lausanne over a 30-year period. 146 charts from consecutive patients with non-syndromic unilateral cleft lip and/or cleft palate who were born between January 1986 and January 2003 were included. RESULTS: The earlier in life a cleft child experience his first otitis media with effusion (OME), the worse his long-term hearing will be. Along with the age of onset of OME, we disclosed an influence of the duration of OME without ventilation tube (VT) insertion on short and long-term hearing outcomes. Different patterns were observed between cleft palate (CP) and cleft lip palate children (CLP), with a higher incidence of otitis media with effusion for the CLP group than the CP group. Direct positive relationship between VT insertion and hearing were disclosed and evaluation of long-term complications did not reveal significant relation with VT insertion. Of note, OME in CLP children led to a higher rate (but not statistically significant) of chronic ear complications than in the CP group, that may indicate more persistent OME or different adverse effect on the middle ear mucosa between CP and CLP children. CONCLUSIONS: Individualized counseling should take into account different factors such as the type of cleft, the age of onset of OME and duration of OME, keeping in mind the adverse effect of persistent middle ear fluid. In the present report, results prone an early ventilation tube insertion to prevent short and long-term injury to the middle ear homeostasis, hearing loss and related issues.

Natural History of Tympanic Membrane Retraction in Children with Cleft Palate.

OBJECTIVES: The natural history of tympanic membrane retraction is unpredictable. To obtain prognostic information for guiding surveillance and treatment, a cohort of children with retraction from cleft palate were prospectively followed for over 5 years. MATERIALS AND METHODS: This was a prospective observational study at a tertiary academic institution. Children with pars tensa retraction were selected from a cohort of 143 children with cleft palate. Thirty-seven ears were assessed with otoendoscopic image capture and audiometry at a median age of 9 years and reassessed at a median follow-up interval of 6.4 years. The severity of tympanic membrane retraction in the serial images of each ear was compared by four pediatric otolaryngologists blinded to the dates of the images. RESULTS: Initially, 19/37 retractions (51%) demonstrated contact with the incus and/or promontory. Follow-up images were rated as stable (n=16) or better (n=12) for 28/37 retractions (76%). Of the nine retractions that became more extensive, two developed cholesteatoma (5% of the total). No ossicular erosion developed in ears without cholesteatoma. Conductive hearing loss (4-tone average air-bone gap >25 decibels hearing level) was initially present in five ears, worsened in one, and normalized without intervention in others. No ears with initial normal hearing developed hearing loss. CONCLUSION: Most tympanic membrane retractions remained stable or improved over time in this cohort of children who were at a risk of persistent eustachian tube dysfunction. Clinically significant progression occurred infrequently, justifying the conservative approach taken to manage these retractions. Such data are necessary to weigh the potential benefit of preventive intervention over observation.

Otologic disorders in Turner syndrome.

INTRODUCTION: Patients with Turner syndrome (TS) have craniofacial malformations, such as Eustachian tube hypoplasia and dysfunction and velar dysfunction, which foster acute otitis media. The aim of this study was to inventory pediatric otologic disorders in patients with TS at their first ENT consultation in our center. PATIENTS AND METHODS: We reviewed the ENT consultation data of pediatric TS patients followed in our center between 2005 and 2015: otoscopy, hearing threshold, and history of acute otitis media or ENT surgery. Data were compared according to karyotype: X monosomy (45,X), mosaic (45,X/46,XX), isochromosome (46,Xi [Xq]), X ring chromosome X (XrX), with Y material, and "other". RESULTS: Ninety patients, with mean age 11.9years (±4.8years) at first ENT consultation, were included: 29% showed tympanic abnormality on otoscopy, 21% had hearing loss, 24% had history of recurrent acute otitis media; 18% had undergone adenoidectomy, 24% T-tube insertion, and 5.6% tympanoplasty. No particular karyotype was associated with higher risk of hearing loss or acute otitis media. CONCLUSION: Patients with TS showed high prevalence of pediatric otologic disorders; they therefore require close and prolonged ENT follow-up.

Assessment of Eustachian tube function in patients with tympanic membrane retraction and in normal subjects / Avaliação da função da tuba de Eustáquio em pacientes com retração de membrana timpânica e em indivíduos normais

Abstract Introduction The diagnosis of Eustachian tube dysfunctions is essential for better understanding of the pathogenesis of chronic otitis media. A series of tests to assess tube function are described in the literature; however, they are methodologically heterogeneous, with differences ranging from application protocols to standardization of tests and their results. Objective To evaluate the variation in middle ear pressure in patients with tympanic membrane retraction and in normal patients during tube function tests, as well as to evaluate intra-individual variation between these tests. Methods An observational, contemporary, cross-sectional study was conducted, in which the factor under study was the variation in middle ear pressure during tube function tests (Valsalva maneuver, sniff test, Toynbee maneuver) in healthy patients and in patients with mild and moderate/severe tympanic retraction. A total of 38 patients (76 ears) were included in the study. Patients underwent tube function tests at two different time points to determine pressure measurements after each maneuver. Statistical analysis was performed using SPSS software, version 18.0, considering p-values <0.05 as statistically significant. Results Mean (standard deviation) age was 11 (2.72) years; 55.3% of patients were male and 44.7% female. The prevalence of type A tympanogram was higher among participants with healthy ears and those with mild retraction, whereas type C tympanograms were more frequent in the moderate/severe retraction group. An increase in middle ear pressure was observed during the Valsalva maneuver at the first time point evaluated in all three groups of ears (p = 0.012). The variation in pressure was not significant either for the sniff test or for the Toynbee maneuver at the two time points evaluated (p ≥ 0.05). Agreement between measurements obtained at the two different time points was weak to moderate for all tests in all three groups of ears, and the variations in discrepancy between measurements were higher in ears with moderate/severe tympanic retraction. Conclusion In this study population, the mean pressure in the middle ear showed significant variation only during the Valsalva maneuver at the first time point evaluated in the three groups of ears. Normal ears and those with mild retraction behaved similarly in all tests. The tested maneuvers exhibited weak to moderate intra-individual variation, with the greatest variation occurring in ears with moderate/severe retraction.

Resumo Introdução O diagnóstico das disfunções da tuba auditiva é essencial para o melhor entendimento da patogênese da otite média crônica. A literatura descreve uma série de testes que avaliam a função tubária; contudo, tais exames são metodologicamente heterogêneos, com diferenças que variam desde os protocolos de aplicação até a padronização dos exames e seus resultados. Objetivo Avaliar a variação na pressão na orelha média em pacientes com retração da membrana timpânica e em indivíduos normais durante os testes de função tubária e também avaliar a variação intraindividual desses testes. Método Estudo observacional do tipo transversal e contemporâneo, no qual o fator em estudo foi a variação na pressão na orelha média durante os testes de função tubária (manobra de Valsalva, Sniff Test e manobra de Toynbee) em indivíduos normais e em pacientes com retrações timpânicas leves e moderadas/graves. Foram incluídos 38 pacientes (76 orelhas). Os pacientes foram submetidos, em dois momentos diferentes, a testes de função tubária para determinar as medidas de pressão após cada manobra. A análise estatística foi feita com o programa SPSS, versão 18.0, e consideramos como estatisticamente significativos os valores de p < 0,05. Resultados A média ± desvio padrão da idade foi de 11 ± 2,72 anos; 55,3% dos pacientes eram do gênero masculino e 44,7% do feminino. A prevalência de curvas timpanométricas do tipo A foi mais alta entre os participantes com orelhas normais e naqueles com retrações leves, enquanto as curvas timpanométricas do tipo C foram mais frequentes no grupo com retrações moderadas/graves. Observamos pressões aumentadas na orelha média durante a manobra de Valsalva no primeiro momento da avaliação nos três grupos de orelhas (p = 0,012). A variação na pressão não foi significativa para o Sniff Test, nem para a manobra de Toynbee nos dois momentos de avaliação (p ≥ 0,05). Consideramos que a concordância entre as determinações obtidas nos dois momentos diferentes foi fraca a moderada para todos os testes nos três grupos de orelhas e as variações em termos de discrepância entre as medidas foram maiores nas orelhas com retrações timpânicas moderadas/graves. Conclusão Na população estudada, a média das pressões na orelha média apresentou variação significante apenas durante a manobra de Valsalva no primeiro momento de avaliação, nos três grupos de orelhas. As orelhas normais e aquelas que apresentavam retração leve se comportaram de maneira similar nos testes. As manobras testadas exibiram uma variação intraindividual fraca a moderada e a maior variação ocorreu nas orelhas com retrações moderadas/graves.

Assessment of Eustachian tube function in patients with tympanic membrane retraction and in normal subjects.

INTRODUCTION: The diagnosis of Eustachian tube dysfunctions is essential for better understanding of the pathogenesis of chronic otitis media. A series of tests to assess tube function are described in the literature; however, they are methodologically heterogeneous, with differences ranging from application protocols to standardization of tests and their results. OBJECTIVE: To evaluate the variation in middle ear pressure in patients with tympanic membrane retraction and in normal patients during tube function tests, as well as to evaluate intra-individual variation between these tests. METHODS: An observational, contemporary, cross-sectional study was conducted, in which the factor under study was the variation in middle ear pressure during tube function tests (Valsalva maneuver, sniff test, Toynbee maneuver) in healthy patients and in patients with mild and moderate/severe tympanic retraction. A total of 38 patients (76 ears) were included in the study. Patients underwent tube function tests at two different time points to determine pressure measurements after each maneuver. Statistical analysis was performed using SPSS software, version 18.0, considering p-values <0.05 as statistically significant. RESULTS: Mean (standard deviation) age was 11 (2.72) years; 55.3% of patients were male and 44.7% female. The prevalence of type A tympanogram was higher among participants with healthy ears and those with mild retraction, whereas type C tympanograms were more frequent in the moderate/severe retraction group. An increase in middle ear pressure was observed during the Valsalva maneuver at the first time point evaluated in all three groups of ears (p=0.012). The variation in pressure was not significant either for the sniff test or for the Toynbee maneuver at the two time points evaluated (p≥0.05). Agreement between measurements obtained at the two different time points was weak to moderate for all tests in all three groups of ears, and the variations in discrepancy between measurements were higher in ears with moderate/severe tympanic retraction. CONCLUSION: In this study population, the mean pressure in the middle ear showed significant variation only during the Valsalva maneuver at the first time point evaluated in the three groups of ears. Normal ears and those with mild retraction behaved similarly in all tests. The tested maneuvers exhibited weak to moderate intra-individual variation, with the greatest variation occurring in ears with moderate/severe retraction.

Electrococleografía extratimpánica en una población normal. Estudio descriptivo / Extra-tympanic electrocochleography in a normal population. A descriptive study

Introducción y objetivos: La electrococleografía extratimpánica es un registro elecrofisiológico que refleja la actividad eléctrica acontecida en la cóclea tras un estímulo sonoro. Se obtiene mediante la aplicación del estímulo en el conducto auditivo externo y el registro de la actividad eléctrica mediante electrodos de superficie. Dada la escasa literatura existente acerca de la exploración electrococleográfica normal en nuestro medio, este estudio busca regularizar los valores obtenidos mediante esta exploración en sujetos sin enfermedad otoneurológica y explicar detalladamente el proceso mediante el cual se obtiene el registro. Métodos: Exploración mediante electrococleografía extratimpánica de 60 oídos sin enfermedad otoneurológica y análisis estadístico de los resultados obtenidos. De ellos, 30 oídos fueron explorados a 90dB mientras que otros 30 oídos fueron estimulados a 80dB. Resultados: Se muestran los valores medios de amplitud y latencia del potencial de sumación y del potencial de acción, así como las latencias medias de la onda I y la onda II. Asimismo, se presenta el cálculo del cociente potencial de sumación/potencial de acción. Obtenidos estos resultados se comparan en función de la intensidad del estímulo, del sexo del paciente, del oído estudiado y del grupo etario. Conclusiones: Este estudio recopila datos sobre la electrococleografía en una población normal. Los valores obtenidos están en el rango de los valores normales de otros países, expuestos en la literatura internacional. Estos datos pueden ser muy útiles como referencia a la hora de valorar exploraciones en pacientes con dolencias que afectan la estructura o la función coclear (AU)

Introduction and objectives: Extra-tympanic electrocochleography is an electrophysiological register obtained after stimulating the cochlea with an audible stimulus. This stimulus is applied using an earphone over the external auditory canal, while the electrical activity is registered by surface electrodes. There are few studies that analyse normal electrocochleography in our environment. Thus, the main objective of our study was to regularize the values obtained with electrocochleography in ears without any otoneurological diseases. We explain in detail the process of obtaining the register. Methods: Sixty healthy ears were studied by extratympanic electrocochleography. Statistical results were analysed. While 30 ears were studied with a stimulus at 90dB, another 30 ears were studied with a stimulus at 80dB. Results: Summating potential and action potential latencies and amplitudes were measured. Summating potential/action potential ratios were calculated. Wave I and wave II latencies were also determined. These results were analysed in function of stimulus intensity, patient gender, patient age group and ear side studied. Conclusions: This study collected extra-tympanic electrocochleography data in a normal population and the results were in the range of other international studies obtained in other countries. These data can be used as a reference to evaluate illnesses that affect cochlear structure or functions (AU)

Decision making in patients with natural myringostapediopexy: A study of the contralateral ear.

Naturally occurring myringostapediopexy frequently results in minimal hearing loss and is asymptomatic. Management decisions in such ears, however, often hinge on an appraisal of evolution toward cholesteatoma. The study of the contralateral ear has been used by our research team to infer the progression of chronic otitis media. This cross-sectional, comparative study describes the clinical findings of the contralateral ear in a series of patients with myringostapediopexy. This study included a historical and current sample of 46 patients divided into a pediatric (≤18 years) and an adult group. Patient distribution according to sex was similar (52.2% male), and 56.5% were adults. Mean conductive hearing loss ranged from 14.1 to 21.2 dB in ears with myringostapediopexy and from 16.0 to 26.6 dB in the contralateral ears according to the frequency assessed. The contralateral ear was normal in only 19.6% of the cases of myringostapediopexy. Central tympanic membrane perforation was found in 6.5% of the cases; perforation-retraction, in 17.4%; moderate or severe retraction, in 28.3%; and cholesteatoma, in 28.3%. The prevalence of cholesteatoma in the contralateral ear in the pediatric and adult groups was not significantly different (p = 0.5; χ(2) test). The presence of significant abnormalities, particularly cholesteatoma, in the contralateral ears suggests a probable unfavorable progression in cases of myringostapediopexy and may influence management decisions.

Otitis media.

Otitis media (OM) or middle ear inflammation is a spectrum of diseases, including acute otitis media (AOM), otitis media with effusion (OME; 'glue ear') and chronic suppurative otitis media (CSOM). OM is among the most common diseases in young children worldwide. Although OM may resolve spontaneously without complications, it can be associated with hearing loss and life-long sequelae. In developing countries, CSOM is a leading cause of hearing loss. OM can be of bacterial or viral origin; during 'colds', viruses can ascend through the Eustachian tube to the middle ear and pave the way for bacterial otopathogens that reside in the nasopharynx. Diagnosis depends on typical signs and symptoms, such as acute ear pain and bulging of the tympanic membrane (eardrum) for AOM and hearing loss for OME; diagnostic modalities include (pneumatic) otoscopy, tympanometry and audiometry. Symptomatic management of ear pain and fever is the mainstay of AOM treatment, reserving antibiotics for children with severe, persistent or recurrent infections. Management of OME largely consists of watchful waiting, with ventilation (tympanostomy) tubes primarily for children with chronic effusions and hearing loss, developmental delays or learning difficulties. The role of hearing aids to alleviate symptoms of hearing loss in the management of OME needs further study. Insertion of ventilation tubes and adenoidectomy are common operations for recurrent AOM to prevent recurrences, but their effectiveness is still debated. Despite reports of a decline in the incidence of OM over the past decade, attributed to the implementation of clinical guidelines that promote accurate diagnosis and judicious use of antibiotics and to pneumococcal conjugate vaccination, OM continues to be a leading cause for medical consultation, antibiotic prescription and surgery in high-income countries.

Developmental mechanisms of the tympanic membrane in mammals and non-mammalian amniotes.

The tympanic membrane is a thin layer that originates from the ectoderm, endoderm, and mesenchyme. Molecular-genetic investigations have revealed that interaction between epithelial and mesenchymal cells in the pharyngeal arches is essential for development of the tympanic membrane. We have recently reported that developmental mechanisms underlying the tympanic membrane seem to be different between mouse and chicken, suggesting that the tympanic membrane evolved independently in mammals and non-mammalian amniotes. In this review, we summarize previous studies of tympanic membrane formation in the mouse. We also discuss its formation in amniotes from an evolutionary point of view.

Favorable prognostic factors for long-term postoperative hearing results after canal tympanoplasty for congenital aural stenosis.

OBJECTIVE: We aimed to determine favorable prognostic factors for long-term postoperative hearing results after canal tympanoplasty for congenital aural stenosis (CAS). STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Canal tympanoplasty for CAS was performed in 25 ears. INTERVENTION: Primary repair of CAS. MAIN OUTCOME MEASURES: The influences of the following factors on the success of surgery were assessed by univariate and multivariate logistic regression analyses: modified Jahrsdoerfer grading system total score; age at surgery; patterns of presentation (whether sporadic or syndromic); presence of external auditory canal (EAC) cholesteatoma; presence of ossicular fixation, including the malleus bar; presence of a partial atretic plate; exposure of the facial nerve at the tympanic portion; type of tympanoplasty; and each component of the modified Jahrsdoerfer grading system. RESULTS: The univariate analysis revealed that the absence of EAC cholesteatoma (p = 0.029) and the presence of a partial atretic plate (p = 0.040) were significant predictive factors for favorable hearing prognosis, whereas the multivariate logistic regression analysis showed that an absence of EAC cholesteatoma was the most significant favorable predictive factor (p = 0.011), followed by anterolateral position of the malleus/incus complex with respect to the stapes as the second-most favorable factor (p = 0.021). CONCLUSION: The absence of EAC cholesteatoma and anterolateral position of the malleus/incus complex with respect to the stapes are considered useful in predicting long-term favorable hearing results after canal tympanoplasty for CAS.

Congenital middle ear abnormalities with absence of the oval window: diagnosis, surgery, and audiometric outcomes.

OBJECTIVE: Congenital absence of the oval window (CAOW) is a rare condition in which the stapes footplate fails to develop, resulting in a significant conductive hearing loss in the affected ear. The purpose of this study was to describe the surgical management and outcomes of patients with CAOW undergoing the oval window drill-out (OWD) procedure. MATERIALS AND METHODS: A retrospective chart review of patients with CAOW between 1996 and 2011 was performed. Clinical data of patients who underwent OWD were collected. Seventy-nine patients (103 ears) were confirmed using exploratory tympanotomy as having congenital stapes anomalies and CAOW without any anomalies of the tympanic membrane and external auditory canal. Demographic data, CT findings, operative findings, complications, and preoperative/postoperative audiometry data of patients who underwent OWD were collected. The preoperative and postoperative audiologic findings were analyzed in 42 patients (56 ears) with complete data. RESULTS: Hearing restoration surgery was aborted for various reasons in 14 cases. Six patients underwent revision operations for worsening hearing after their first surgery. The average preoperative 4 tone air conduction threshold was 67 dB; the average 6-month postoperative four tone air conduction threshold was 49 dB, and the average postoperative hearing gain was 18 dB. For the 56 ears, the average 4 tone air conduction threshold 6 months after surgery was significantly lower than the preoperative threshold. CONCLUSION: The oval window drill-out procedure is a viable operation for patients with congenital absence of the oval window, and it is important for surgeons to develop personalized treatment programs to improve patients' hearing with minimal complications.

Membranous band between the tympanic membrane and the external auditory canal.

Two cases of a membranous band between the tympanic membrane and the external auditory canal are presented. These characteristic structures are rare, but observed in several first branchial cleft anomalies. Neither patient presented with an infection. In Case 1, an eleven-year-old girl has this structure in her right ear. In Case 2, a thirteen-year-old boy has this structure in his left ear. Both patients demonstrated slight air-bone gaps in the affected ear with a pure-tone audiometric test, due to limited vibrations of the tympanic membrane.

The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia.

CONCLUSION: The extent of inferior displacement of the mastoid tegmen is related to the severity of congenital aural atresia (CAA). OBJECTIVE: To analyze anatomic variations observed on high-resolution temporal bone computed tomography (TBCT) in patients with CAA, the extent of inferior displacement of the mastoid tegmen and the size of the incudo-stapedial (IS) joint angle were compared with surgical parameters for atresiaplasty, such as Jahrsdoerfer score and hearing acuity. METHODS: Sixty-one patients with unilateral CAA underwent high-resolution TBCT and hearing tests. We evaluated TBCTs in terms of Jahrsdoerfer criteria and analyzed the relationships among the inferior displacement of the mastoid tegmen, size of the IS joint angle, hearing acuity, and the Jahrsdoerfer score on the atretic side. RESULTS: IS joint angle on the atretic side was found to be 120.6 ± 11°, which was significantly greater than the corresponding value of 103.4 ± 5.4° on the normal side. Cholesteatoma occurred significantly more often in CAA patients with narrow external auditory canals (EACs) (9/27; 33.3%), compared with CAA patients with no EAC opening (2/34; 5.9%). There was a significant relation between the extent of inferior displacement of the mastoid tegmen (28.9% in the atretic side and 17.3% in the normal side) and the Jahrsdoerfer score (p < 0.0001).

Supermicrosurgical reconstruction for congenital aural atresia using a pure skin perforator flap: concept and long-term results.

BACKGROUND: Several surgical methods for creating an external auditory canal have been developed in step with clinical innovation. However, revision surgery is often required to address complications, which include external auditory canal stenosis, lateralization of the tympanic membrane, and chronic recurrent otorrhea. These complications occur frequently within 6 to 12 months after surgery. To decrease the incidence of complications, the authors reconstruct the external auditory canal and tympanic membrane in patients with congenital aural atresia using a free pure skin perforator flap that is as thin as a skin graft. They report their outcomes for hearing acuity and complications over 12 months after the operation. METHODS: The authors performed reconstruction of the external auditory canal and tympanic membrane in nine patients with congenital aural atresia. The flap design was based on a pure skin perforator derived from the groin area in eight patients. The flap was folded into an approximately 1.5×3-cm sac and inserted into the external auditory canal. Anastomosis was performed between the perforator vessels and superficial temporal vessels. RESULTS: All pure skin perforator flaps survived, although one case had partial epidermal necrosis. The audiologic follow-up period ranged from 12 to 24 months (mean, 17 months). The mean pure-tone average was 65.1 dB (range, 53 to 80 dB) preoperatively and improved to 32.4 dB (range, 8 to 53 dB) postoperatively. None of these cases showed any potential complications. CONCLUSION: This flap may therefore reduce complications and help to maintain hearing acuity in the long term. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Myringostapediopexy: is it a natural type III tympanoplasty?

INTRODUCTION: Natural myringostapediopexy is an infrequent abnormality, and studies about resulting hearing loss are scarce. In several cases, natural myringostapediopexy may function as a Type III tympanoplasty. OBJECTIVE: This study evaluated conductive hearing loss in myringostapediopexy. MATERIALS AND METHODS: This cross-sectional comparative study included a historical and current sample of 46 patients, one with bilateral myringostapediopexy, at a total of 47 ears. All underwent pure tone and speech audiometry and were divided according to age into a pediatric (younger than 18 yr) and an adult group. The Statistical Package for Social Science (SPSS) 10.0 was used for statistical analysis, and the level of significance was set at p lower than 0.05. RESULTS: Patient distribution according to sex was similar (53.2% male), and 57.4% were adults. Mean conductive hearing loss in ears with myringostapediopexy ranged from 14.13 to 21.28 dB according to the frequency assessed. Pure tone average was 18.46 dB. A conductive hearing loss equal to or lower than 25 dB at all frequencies was found in 53% of the patients. The 2,000 and 3,000 Hz frequencies had the greatest prevalence of clinically nonsignificant conductive hearing loss (87% and 91%). Sensorineural hearing loss was found in 14 patients (30%), all adults, and 43% of the cases were mild. The comparison according to age did not reveal any significant differences in conductive hearing loss at any of the frequencies. CONCLUSION: Most patients with natural myringostapediopexy included in the study had clinically irrelevant conductive hearing loss. There were no differences in conduction loss between children and adults. The reconstruction of the ossicular chain and tympanoplasty, for purely functional reasons, are not justified in these cases, particularly not for patients with mixed hearing loss.

[The early diagnostics of retraction pockets of the tympanic membrane in children].

The most important literature data concerning retraction pockets (RP) of the tympanic membrane and their currently accepted classification are presented. The objective of the present work was to develop criteria for the objective estimation of the dynamic state of the tympanic retraction pockets in children presenting with non-perforating forms of otitis media. A total of 138 children suffering from exudative otitis media were available for observation; retraction pockets were found in the majority of these patients. Otomicroscopic characteristics of various RP species are described. A diagnostic approach to the observation of the dynamic state of the tympanic retraction pockets is proposed. The presence of the retraction pockets of the tympanic membrane is considered to be a risk factor of the development of cholesteatoma and chronic purulent pathology of the middle ear in the children. Deep retraction pockets without a controllable bottom and attic cholesteatomas were identified in 16 (11.6%) and 6 (4.3%) of the examined children respectively. They were treated by means of sparing otosurgery.

Otologic and audiologic features of ethnic Chinese patients with Turner syndrome in Taiwan.

BACKGROUND/PURPOSE: Otologic and audiologic characteristics of Turner syndrome (TS) have been well documented in Caucasian-but not in Asian-populations. We report these features and possible causative factors for hearing loss in ethnic Chinese TS patients in Taiwan. METHODS: The study was a cross-sectional trial that analyzed patients diagnosed with TS. We enrolled patients for otologic evaluations and age-appropriate pure tone audiometry. To explore the potential associations with hearing loss, we studied patient karyotype, history of recurrent otitis media (OM) and various craniofacial anomalies. RESULTS: The 46 patients (mean age 17.3 years, range 5-34 years) enrolled, 22 (47.8%) had a history of recurrent OM. Otoscopic examination identified eardrum abnormalities in 45.6% of patients with myringosclerosis as the most common condition. A total of 21.7% patients showed auricular anomalies. Audiologic analysis revealed five cases (10.7%) with conductive hearing loss (CHL) and eight cases (17.4%) with sensorineural hearing loss (SNHL). Among patients with a history of recurrent OM, CHL prevailed (p = 0.0192) over SNHL (p = 0.1278). Karyotype and craniofacial anomalies were not associated with CHL or SNHL (p > 0.05). CONCLUSION: We found varying degrees of otologic and audiologic abnormalities among the TS in ethnic Chinese population. About one-half of the patients had recurrent OM, which was more likely to be associated with CHL in early life. Therefore, regular surveillance, early diagnosis, and the initiation of appropriate treatment are crucial in improving the hearing and speech in children with TS as well as in preventing short- and long-term associated complications.