First branchial cleft cyst accompanied by external auditory canal atresia and middle ear malformation:A case report

BACKGROUND We report a rare case of first branchial cleft anomaly(FBCA)accompanied by bony atresia of the external auditory canal,middle ear malformation,and location malformation of the facial nerve according to the intraoperative findings.CASE SUMMARY A 19-year-old male patient presented to our department with a mass behind the right earlobe and recurrent postauricular swelling and pain since childhood,he also had severe hearing loss in the right ear since birth.The patient underwent surgery including mass removal,mastoidectomy,and simultaneous meatoplasty and ossiculoplasty under microscopy.No facial palsy or recurrence was noted during postoperative follow-up.CONCLUSION FBCAs are rare,and to our knowledge,this is the first report of FBCA accompanied by external auditory canal bony atresia,middle ear malformation,and location malformation of the facial nerve.An effective postauricular approach under microscopy facilitated complete lesion removal and simultaneous otologic reconstruction.

Chromsome and pedigree analysis of congenital malformation of external and middle ear

Objective:Using chromosome analysis of a Chinese pedigree to investigate the genetic relationship between chromosome and congenital malformation of the external and middle ear combined with pre-auricular fistula.Methods: Clinical data of all members from 3 family with congenital malformation of the external and middle ear combined with pre-auricular fistula were collected. A pedigrees chart of each family was draw Venous blood samples of all participants were taken for karyotype analysis.Results: A total of 21 members in three generations from three families were screened. Chromosomal abnormalities were detected in 9 people, including 3 patients and 6 carriers. The carriers were fathers and paternal grandparents. Structural abnormalities in chromosomes 1, 2 and 11 were identified.Conclusion:The genetic patterns of congenital malformation of the external and middle ear combined with the pre- auricular fistula in these three families are consistent with autosomal recessive inheritance. Family survey is helpful for further study of this disease.

Major Ear Aplasia and Cochleovestibular Dysplasia: Rare Congenital Malformation about a Case

Introduction: The congenital malformations of the ear are rare and often isolated, may be unilateral or bilateral, and can be associated with another syndromic malformation. Such malformations so not necessarily impact aesthetics and social relations. Case Presentation: The authors report the case of Samuel M, male born at 38 WA, who is the first child of healthy parents from the same socio-cultural area. His birth weight was 2800 g and he did not have any risk factors for deafness or concept of obstetrical trauma. He presented with congenital malformation manifesting as bilateral ear aplasia with unilateral facial paralysis. Computed tomography revealed abnormalities of the inner ear;functional explorations, such as PEA and OEA, showed findings in favor of bilateral cophosis. ASSR (Auditory Steady-State Responses) was not performed. The announcement of the serious diagnosis of deafness requires multidisciplinary care in order to plan a therapeutic program to limit the impact on the development of language, schooling, and consequently, the socio-professional future of children. Conclusion: This clinical case underlines the fact that interest of the clinical interview before possible multiple surgery does not always guarantee the satisfaction of the desire for repair in this type of patient.

A child with severe inner ear malformations with favorable hearing utilization and balance functions after wearing hearing aids

Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations(right side, incomplete partition type I [IP-I]; left side, common cavity deformity). Hearing aids had a remarkable effect in this patient, enabling sufficient and favorable hearing recovery such as to allow the patient to engage in daily conversations. Per-rotatory nystagmus was recorded on an electronystagmogram for both right and left rotations in a damped rotational chair test. It is rare for deaf children with severe bilateral inner ear malformation to demonstrate favorable development in hearing and good equilibrium function. Our findings suggest that auditoryevestibular hair cells in this patient may have been partially preserved despite IP-I in the right ear and common cavity deformity of the left ear.

Application of high resolution computer tomography in external ear canal cholesteatoma diagnosis

Objective: To evaluate High Resolution Computer Tomography(HRCT) in the diagnosis of external ear canal cholesteatoma.Methods: In this retrospective study, HRCTs of 27 patients with external ear canal cholesteatoma were reviewed. The changes in the external ear canal, tympanic membrane(TM), scutum, tympanum and mastoid were measured and categorized.Results: Fourteen patients showed no or mild destruction in the external ear canal(stage Ⅰ group). Eight patients had obvious enlargement in the external ear canal(stage Ⅱ group) but showed limited destructions of the mastoid bone and no damage of the tympanums. Five patients had serious destruction of the mastoid bone and damage of the tympanum(stage Ⅲ group). All patients in the stage Ⅲ group showed a compression of manubriums and TMs, with 3 having damages on ossicular chain. Bone destruction of the vertical section of facial nerve canal was discovered in one case in the stage Ⅲ group.Conclusion: HRCT can provide detail information about the extent of external ear canal cholesteatoma. Such information can be used to identify special situations with serious complications and to differentiate external ear canal cholesteatoma from middle ear cholesteatoma.

Capillary Hemangioma of the Middle Ear and External Auditory Canal:a Case Report

We report a case of capillary hemangiomsa that involved the entire middle ear space,external auditory canal(EAC) and tympanic antrum.Symptoms in the case included ear fullness,hearing loss,otalgia and otorrhea.The case was misdiagnosed as recurrent chronic otitis media with granulation preoperatively.A diagnosis of capillary hemangioma was established by postoperative histological examination.The management of capillary hemangioma of the middle ear and external auditory canal is discussed,with a review of the literature.Because of its variable and sometimes misleading clinical presentation,hemangioma can initially be misdiagnosed as other lesions.Therefore,a high index of suspicion is necessary for early and accurate diagnosis.

Squamous Cell Carcinoma of the External Ear in a Child

Tumors involving the head and neck are uncommon in children. Furthermore, those which involve the external ear are extremely rare. In the external ear itself, the most commonly encountered malignancy is Squamous Cell Carcinoma, both in the adult and pediatric age groups. We encountered one such case of a 14 years old male with a recurring skin lesion involving the right external ear. In this report, we wish to highlight and address the difficulty in recognizing and managing such an unwonted pathology as Squamous Cell Carcinoma in the pediatric age group.

Survival in Primary Carcinoma of the External and Middle Ear Is Strongly Dependent on Stage at Diagnosi

Conclusion: Early-stage detection of temporal bone carcinoma improves the survival rate. When detected at an advanced stage, predicted prognosis is poor even with a combination of surgery, radiotherapy and chemotherapy. Objectives: We evaluated treatment strategies and survival rates in cases of carcinomas of the external and middle ear. Method: Retrospective review of patients treated at Ehime University Hospital during the 32 years from 1977 to 2008. Subjects were 41 patients, consisting of 21 men and 20 women with a mean age of 63.2 years. Modified Pittsburgh staging system: stage I in 11 cases (27%), stage II in 4 (10%), stage III in 6 (15%) and stage IV in 20 (49%). Histopathology: squamous cell carcinoma (SCC) in 35 cases, adenoid cystic carcinoma (ACC) in 3, and adenocarcinoma (AC) in 3. Thirty-eight patients were treated by surgery. Radiation was added postoperatively in 23 patients. Three patients were treated by chemoradiotherapy. Results: The survival rate of carcinomas detected at an advanced stage was poor with a disease-specific 5-year survival rate of 100% in early-stage disease (stage I and II) versus 20.8% in stage III and 27.5% in stage IV disease, irrespective of histopathology of the tumor.

Middle ear foreign body causing cholesteatoma and external auditory canal granuloma: a case report

Objective Patients with unilateral ear discharge and hearing loss often have external or middle ear diseases. We present a 55-year-old man who suffered from persistent ear discharge and hearing loss in the left ear. Local findings showed that his left ear canal was filled with a large amount of granulation tissue, with purulent, foul-smelling discharge. Computed tomography indicated left middle ear cholesteatoma and mass shadow in the left external auditory canal. Modified radical mastoidectomy was performed. A piece of white plastic stick was found in the middle ear during the operation. Foreign body-induced cholesteatoma and external auditory canal granuloma in adults are very rare. We present this rare case so that these conditions can be better recognized and understood.

Communication outcomes following cochlear implantation in a child with cystic cochleovestibular anomaly

Cochlear implantation is one of the best amongst the various management options available for children and adults with severe to profound sensorineural hearing loss.Inner ear and internal auditory canal(IAC) malformations accounts to approximately 25% of congenital sensorineural hearing loss in children.The primary goal of this report was to evaluate the communication outcomes after cochlear implantation in a child with cystic cochleovestibular anomaly(CCVA).The child was evaluated through various standardized outcome measures at regular intervals to track the progress in terms of auditory and spoken language skills.The scores on Categories of Auditory Perception(CAP),Meaningful Auditory Integration Scale(MAIS),Speech Intelligibility Rating(SIR),Meaningful Use of Speech Scale(MUSS),and listening and spoken language skills showed a significant leap in 12 months duration post implantation.The report thus highlights and correlates the significant progress in auditory and spoken language skills of the child with congenital malformations to appropriate auditory rehabilitation and intensive parental training.

Role of the mental foramens in dolphin hearing

The role of mental foramens in dolphin hearing was studied in the present work. To this effect the mental foramens’ morphology features which are essential from acoustical viewpoint have been studied. The patterns of relationship between the location of mental foramens and their sizes are found. The affinity of the mental foramens’ morphology and acoustics that the nature had created testify acoustical expediency of the mental foramens’ architecture. This natural inference in the main is confirmed in this work by the experimental data. The mean values of detection thresholds of short broadband stimuli with spectral maximum on frequencies 8, 16, 30 and 100 kHz at acoustical shielding the mental foramens increased on 30, 34, 40 and 50 dB, respectively. Results obtained testify that the mental foramens are the unique sound-conducting pathway into the fat body of the mandibular canal for the sounds of all frequencies used in the experiment, approximately 6?150 kHz (in view of stimuli broadbandness). The left and right row of the mental foramens together with respective mandibular canal plays the role of pinna and external auditory meatus if to use conventional terminology of a land mammal ear. But it is already qualitatively the new external ear implemented by the nature as the receive array and acoustical horn. The new external ear has apparently appeared in result of the dolphins’ ancestors’ adaptation to new environment conditions, as evolutionary adaptation of the ear to the water and as functional adaptation of the ear in order that to fulfill the new more sophisticated functions in the structure of sonar. The findings give good reason to suppose existence of the same external ear in Odontoceti.

Treatment of Hordeolum by Bloodletting at Ear Apex

Hordeolum, the acute pyogenic infection of staphylococci in the palpebral glands, is one of the most commonly seen disorders of the external eye diseases. According to the location of the lesion, it can be divided into internal and external hordeolum. The early symptoms are redness, swelling, and pain in the eyelid. In severe cases, the condition can be accompanied with ipsilateral headache and swelling of the pre-auricular lymph node. Since TCM bloodletting has remarkable effect for treating hordeolum at the early stage, we would like to recommend this simple, easy and effective treatment to doctors who are interested in physiocracy.

A CLINICAL OBSERVATION ON CORONARY HEART DISEASE TREATED BY OTOPUNCTURE AT HEART OTOPOINT

In the clinical study of acupuncture for treatment of coronary heart disease, body acupuncture was primarily used, and otopuncture was seldom used. Under the premise of guarantee of therapeutic effects, screening out relatively specific acupoint for treatment of coronary disease is a

Phosphatidylinositol 4-kinase β mutations cause nonsyndromic sensorineural deafness and inner ear malformation

Congenital hearing loss is a common disorder worldwide.Heterogeneous gene variation accounts for approximately 20-25%of such patients.We investigated a five-generation Chinese family with autosomaldominant nonsyndromic sensorineural hearing loss(SNHL).No wave was detected in the pure-tone audiometry,and the auditory brainstem response was absent in all patients.Computed tomography of the patients,as well as of two sporadic SNHL cases,showed bilateral inner ear anomaly,cochlear maldevelopment,absence of the osseous spiral lamina,and an enlarged vestibular aqueduct.Such findings were absent in nonaffected persons.We used linkage analysis and exome sequencing and uncovered a heterozygous missense mutation in the PI4 KB gene(p.Gln121 Arg)encoding phosphatidylinositol 4-kinaseβ(PI4 KB)from the patients in this family.In addition,3 missense PI4 KB(p.Val434 Gly,p.Glu667 Lys,and p.Met739 Arg)mutations were identified in five patients with nonsyndromic SNHL from 57 sporadic cases.No such mutations were present within 600 Chinese controls,the 1000 genome project,gnom AD,or similar databases.Depleting pi4 kb m RNA expression in zebrafish caused inner ear abnormalities and audiosensory impairment,mimicking the patient phenotypes.Moreover,overexpression of 4 human missense PI4 KB mutant m RNAs in zebrafish embryos resulted in impaired hearing function,suggesting dominant-negative effects.Taken together,our results reveal that PI4 KB mutations can cause SNHL and inner ear malformation.PI4 KB should be included in neonatal deafness screening.

Solitary Neurofibroma of External Aural Canal

Neurofibroma is a benign tumor of the connective tissue of the peripheral nerves, developed mainly at the endoneurium. The most common localizations are the extremities of the limbs and the head and neck region. Neurofibromas are often associated with neurofibromatosis type 1. An isolated localization in the auditory canal is exceptional. We report the case of a 45-year-old female patient presenting with a neurofibroma of the auditory canal which had previously caused hearing loss. Examination revealed a tissue mass firm, painless and covered with normal skin obstructing the external auditory canal. The patient’s skin examination revealed no café-au-lait spots. A CT scan of the ear showed a hypodense tissue mass. Surgical removal via the ear canal gave good results. In conclusion, an isolated neurofibroma of the external auditory canal is a rare benign tumor with a good prognosis. The surgical approach depends on the exact location of the mass and the surgeon’s experience.

Nodular fasciitis of the external auditory canal: Clinical case report and review of the literature

Nodular fasciitis is a benign reactive soft tissue tumor arising from fibroblasts and myofibroblasts.Its incidence is low and misdiagnosis is frequent especially for malignant lesions.This can lead to inappropriate and unnecessary invasive treatment.Nodular fasciitis of the external auditory canal is extremely rare.So far,around fifteen cases have been reported.We present here the case of a 90-year-old patient with nodular fasciitis of the right external auditory canal.The lesion extends anteriorly for 6.5 cm and reaches the posterior wall of the maxillary sinus.To our knowledge,this is the first case in the literature of an external auditory canal nodular fasciitis presenting as an inflammatory ear polyp with such a wide extension.

Challenges of Management of Arteriovenous Malformation of the Pinna in a Low Resource Setting: A Case Report

Arteriovenous Malformation (AVM) is a lesion of vascular development characterized by an abnormal communication between an artery and a vein without an intervening capillary bed. AVM of the pinna is very rare with very few reported cases in Africa. Management of an AVM is often daunting, most especially in a resource challenged centre like ours where facilities for super selective embolization are not available. We present the case of a 17-year-old female with a pulsatile swelling of the left pinna since childhood. Clinical and radiological diagnosis of arteriovenous malformation was made. She was managed surgically with left external carotid artery ligation, partial auricular resection and postauricular scalp rotation flap cover of the cartilage remnant. The aetiopathogenesis, clinical presentation, diagnostic confirmation and treatment options are discussed. We also discuss the challenges of managing AVM in poor resource settings like ours.

Keeping an ear out:size relationship of the tympanic bullae and pinnae in bandicoots and bilbies(Marsupialia:Peramelemorphia)

Bandicoots and bilbies(Order Peramelemorphia)occupy a broad range of habitats across Australia and New Guinea,from open,arid deserts to dense forests.This once diverse group has been particularly vulnerable to habitat loss and introduced eutherian predators,and numerous species extinctions and range retractions have occurred.Understanding reasons for this loss requires greater understanding of their biology.Morphology of the pinnae and tympanic bullae varies markedly among species.As hearing is important for both predator avoidance and prey location,the variability in ear morphology could reflect specialization and adaptation to specific environments,and therefore be of conservation relevance.We measured 798 museum specimens representing 29 species of Peramelemorphia.Controlling for phylogenetic relatedness and head length,pinna surface area was weakly negatively correlated with average precipitation(rainfall being our surrogate measure of vegetation productivity/complexity),and there were no environmental correlates with effective diameter(pinna width).Controlling for phylogenetic relatedness and skull length,tympanic bulla volume was negatively correlated with precipitation.Species that inhabited drier habitats,which would be open and allow sound to carry further with less obstruction,had relatively larger pinnae and tympanic bullae.In contrast,species from higher rainfall habitats,where sounds would be attenuated and diffused by dense vegetation,had the smallest pinnae and bullae,suggesting that low-frequency hearing is not as important in these habitats.Associations with temperature did not reach statistical significance.These findings highlight linkages between hearing traits and habitat that can inform conservation and management strategies for threatened species.

新生儿耳廓畸形筛查及耳模无创矫正疗效评估

目的通过新生儿查体时行外耳廓检查,统计分析新生儿耳廓畸形的发生率、矫正率、并发症发生率,评估耳模无创矫正治疗效果。方法选取2020年1月1日—2021年7月1日淮北市人民医院出生的新生儿3797例(7594耳),其中耳廓畸形患儿616例(851耳),将进行治疗的119例(173耳)患儿根据出生时间分为1组(≤7 d)125耳、2组(8~42 d)31耳,3组(>42 d)17耳。对于轻微不需治疗及拒绝治疗的患儿监护人,分别于出生后7、14 d进行电话随访,收集相关数据进行汇总分析。比较3组治疗总有效率、并发症发生率、治疗时间。结果新生儿耳廓畸形发生率为11.21%(851/7594),19.32%(119/616)的新生儿耳廓畸形接受了无创矫正治疗,矫正率为20.33%(173/851),12例(10.08%)患儿在矫正过程中出现湿疹,3耳中途放弃治疗,2耳效果不佳,治疗总有效率为97.11%(168/173)。1组治疗有效率显著高于2组和3组,差异有统计学意义(P<0.01);2组与3组治疗有效率比较,差异无统计学意义(P=0.225)。1组治疗时间短于2组和3组,差异有统计学意义(P<0.01);2组治疗时间短于3组,差异有统计学意义(P<0.01)。1组并发症发生率低于2组和3组,差异有统计学意义(P<0.01);2组并发症发生率低于3组,但差异无统计学意义(P=0.530)。结论新生儿耳廓畸形发生率较高,耳模型无创矫正效果好,在新生儿查体时即可对耳廓畸形做出筛查,在最早期发现患耳、避免漏诊,越早治疗效果越好。

Recurrent glomangioma("true"glomus tumor)of the middle ear and mastoid

Objective:To review current literature and experience with glomangiomas,or true glomus tumors of the middle ear and mastoid as well asto report on the exceptionally rare case of a glomangiomastemming from the middle ear space with multiple recurrences.Methods:Review of existing world literature and description of personal experience with rare cases of a glomangioma of the middle ear and mastoid.Results:Review of existing literature revealed two cases of patients presenting with tinnitus and hearing loss refractory to medical management.Both patients were ultimately diagnosed with glomangioma on histopathology.Complete surgical excision is thought to be curative.Patient:A 36-year-old woman presented with a rare case of a glomangioma of the middle ear presenting with unilateral hearing loss.She was noted to have a mass behind the tympanic membrane.Imaging revealed a diffuse mass filling the mastoid air cells.Imaging characteristics and histology were consistent with a glomangioma.Intervention:Initial resection via mastoidectomy using a postauricular approach.The tympanic membrane was reconstructed with temporalis tissue.Follow-up revision tympanomas-toidectomy was performed upon recurrence of disease.The chorda tympani were sacrificed due to tumor involvement.The incus and head of the malleus were removed to gain better access to the tumor.The ossicular chain was reconstructed with a Goldenberg Total Ossicular Prosthesis.Main outcome measure:Recurrence of disease.Follow-up:In the 67 months since her most recent surgery,there has been no evidence of recurrence by CT or physical exam.Conclusion:Glomangioma of the middle ear represents an exceptionally rare entity that can present in a similar fashion to a paraganglioma.