Narrow External Auditory Canal Research Articles

Hearing Improvement in Tympanoplasty, with and Without Canalplasty.

Primary objective of this study was to compare the role of canalplasty in tympanoplasty, with that of only tympanoplasty, in patients of chronic suppurative otitis media with narrow external auditory canal and moderate to large central perforation, in terms of hearing improvement, graft uptake, intra operative ease. This study included 60 patients with chronic mucosal otitis media with narrow external auditory canal, with moderate to large central perforation, presenting to our institution from September 2019 to August 2021. Group A consisted of 30 patients, who underwent tympanoplasty with canalplasty and Group B consisted of 30 patients, who underwent tympanoplasty without canalplasty. Both the groups were followed up for 3months, compared and analysed for hearing improvement and graft uptake. The results of our study indicated that Group A achieved 93.3% graft uptake rates compared to group B which achieved 80%. In Group A gain in air bone gap was 12.43dB, whereas in Group B it was about 9.50dB. Group A had significant hearing improvement and better graft uptake compared to Group B. It is advantageous to perform canalplasty prior to tympanoplasty in patients with narrow external auditory canal in whom the entire rim of annulus is not visible in one microscopic view. It yields better hearing improvement and graft uptake and prevents lateralization of the graft.

Auditory and Language Features in Children with 18q Deletion: From Diagnosis to Treatment

Introduction: 18q deletion is a rare genetic condition which occurs approximately 1 in 40,000 live births. Site and size of mutation on DNA may be very different among patients. The aim of this study is to describe three cases of 18q, analyzing their auditory and language skills. A secondary aim is to compare our results with those reported in the literature in order to propose a standardized assessment protocol and guidelines for auditory treatment and rehabilitation. Method: We describe the clinical cases of three pediatric patients with 18q deletion. All children underwent detailed audiological evaluations, including otoscopy, Pure Tone Audiometry (PTA) and Auditory Brainstem Responses (ABRs). We also administered them language and speech tests, depending on children age and skills. We propose different solutions for auditory rehabilitation and we compare our patient’s outcomes with those described in the literature. Results: All children showed hearing and narrow External Auditory Canals (EACs). Type of auditory rehabilitation changed among patients. Comorbidities and severity of the disease varied according to the genetic mutation. Data from the literature review are consistent with our findings. Conclusion: Despite the heterogeneity of phenotypes and severity, hearing loss and stenotic EACs were detected in all our patients with 18q deletion. Accurate diagnosis and follow-up are mandatory to improve auditory and language skills and quality of life. Due to the complexity of the cases, no standardized assessment protocols or treatment could be routinely proposed.

An observational study of endoscopic versus microscopic type I tympanoplasty in tubotympanic chronic otitis media in a tertiary health care centre

<p class="abstract"><strong>Background:</strong> Chronic otitis media (COM) is a widespread disease in the developing countries. It is treated by tympanoplasty, traditionally performed with the microscope which limits the visual field in the deep recesses of the middle ear, especially in narrow external auditory canal. Using endoscope has minimised these difficulties by providing a wide field view with angled magnification, reducing the duration of surgery. The advantages and disadvantages of both techniques is evaluated in our study.</p><p class="abstract"><strong>Methods:</strong> In this observational study, 60 patients of tubotympanic COM between 12-60 years of age with small to medium sized central perforation and air-bone gap of less than 45 decibel were included. They were divided into two groups (group A and group B) of 30 patients each. Group A underwent microscopic type 1 tympanoplasty and group B underwent endoscopic type 1 tympanoplasty. The duration of the surgery, the post-operative improvement in hearing and the graft uptake was analysed after 3 months of surgery. The post-operative hospital stay duration was noted. </p><p class="abstract"><strong>Results:</strong> The post-operative hearing improvement and the graft uptake after 3 months of the surgery was statistically not significant between the two groups. But the mean duration of the operation and the post-operative hospital stay was significantly lower in the endoscopic group with a p value <0.05.</p><p class="abstract"><strong>Conclusions:</strong> Endoscopes are better in otological procedures as it reduces the surgical duration, provides better visibility, lessens the post-operative hospital stay, reducing the economic burden of the patient.</p>

The MO-meatocanalplasty: long-term results in the narrow external auditory canal with recurrent otitis externa or the inability to wear a hearing aid.

The MO-meatocanalplasty is the oblique modification of the M-meatoplasty. The MO-meatocanalplasty was designed to address the superior quadrants of the meatus and the bony canal without the need for a retro-auricular incision. This retrospective analysis was performed to evaluate the long-term results of the MO-meatocanalplasty in patients with a narrow external auditory canal (EAC) with recurrent otitis externa or in patients unable to wear a hearing aid. Twenty-two ears in twenty consecutive patients who received a MO-meatocanalplasty for a narrow EAC with recurrent otitis externa or the inability to wear a hearing aid were analysed retrospectively. There were no patients included with any type of previous or planned second stage tympanoplasty procedures. A follow-up period of 3years was analysed for postoperative recurrent narrowing, the self-cleaning capacity of the EAC, the recurrence of otitis externa, the inability to wear a hearing aid, change in hearing level and for all types of aesthetical complaints. The MO-meatocanalplasty procedure was effective in 82% (n = 18). Postoperative recurrent narrowing was detected in 9% (n = 2). Insufficient self-cleaning capacity of the EAC was 9.1% (n = 2). The ability to wear a hearing aid was restored in all patients with the need for a hearing aid. No aesthetical complaints were reported. The MO-meatocanalplasty is an effective, safe and aesthetical accepted procedure to address the narrow meatus and external auditory canal. With this procedure, there is no need for a retro-auricular incision in order to create a well aerated, dry and self-cleaning EAC in patients with a narrow EAC with recurrent otitis externa or in patient with the inability to wear a hearing aid.

Repair effect of xenogeneic acellular dermal matrix during external auditory canal reconstruction after canal wall down mastoidectomy

Background: In some cases, there is insufficient external auditory canal (EAC) skin to cover the reconstructed canal wall after canal wall down (CWD) mastoidectomy. Acellular dermal matrix (ADM) can help to repair the skin, mucosa and other epidermal tissue defects.Aims: To investigate the repair effect of xenogeneic ADM (xeno-ADM) for EAC skin defects.Material and Methods: We retrospectively analyzed 28 patients who underwent open mastoidectomy combined with canal wall reconstruction and mastoid obliteration in our hospital. The xeno-ADM was used to repair the EAC skin defect. The epithelialization time, dressing change times, complications and hearing improvement post-operation were summarized and analyzed.Results: Reasons for using xeno-ADM included: 11 (39.3%) cases suffered from extensive middle ear cholesteatoma, three (10.7%) cases suffered from advanced EAC cholesteatoma, eight (28.6%) cases to solve the cavity-related problems, and six (21.4%) patients had a narrow EAC. The postoperative epithelialization time was 5.8 ± 1.6 weeks, and the number of dressing changes was 4.8 ± 1.6 times. There was no xeno-ADM rejection and related complications, the postoperative hearing improvement was statistically significant (p = .013).Conclusions and Significance: Xeno-ADM could be a safe, effective and simple method for repairing skin defect in the reconstruction of EAC after CWD mastoidectomy.

How Palliative Care changed a Child’s Life with a Very Rare Disease: A Case with Cerebrocostomandibular Syndrome

Case Description: We present a 9-year-old female with Cerebrocostomandibular syndrome (CCMS), restrictive lung disease, chronic respiratory failure, failure to thrive, GERD, hearing loss, bilateral cholesteatoma and severe scoliosis, born at 31 weeks of gestational age via emergency C-section due to lack of fetal movement. Prenatal ultrasound was concerning for a cystic hygroma and micrognathia. NICU course was characterized by respiratory distress requiring intubation. Consistent with CCMS, physical findings significant for micrognathia, scapular and vertebral anomalies and rib dysplasia were noted as well as narrow external auditory canals and …

NIEKTÓRE PRZYCZYNY NIEPOWODZEŃ CHIRURGICZNEGO LECZENIA OTOSKLEROZY NA PODSTAWIE OPISU PRZYPADKU

IntroductionSurgical treatment of otosclerosis requires considerable experience of the otosurgeon, especially in advanced cases or in an ear with an unusually narrow external auditory canal, a narrow niche of the round window, a significantly thickened stapes footplate, or an exposed or hanging facial nerve. Case involving subsequent reoperations by different surgeons can be particularly difficult. Collective knowledge of treatment failures during otosclerosis reoperation, as set out in the literature, does not list the difficulties encountered, fails to assess most causes of failure, and does not describe remedies and corrective techniques.Material and MethodsThe course of treatment followed by a 55-year-old complaining of progressive, bilateral, mixed hearing loss due to otosclerosis is described. The patient’s treatment consisted of stapes mobilization, followed by stapedotomy and restapedotomy of the right ear, and stapetodomy of the left ear.ResultsThere was short-term improvement of hearing after mobilization of the stapes, but its subsequent deterioration, with elevation of both the air- and bone-conduction thresholds in the right ear, were indications for reoperation and stapedotomy. Short-term improvement in hearing was achieved after the operation, followed by rapid hearing loss. Due to this and growing balance disorders after nearly a year, a restapedotomy was performed with complete closure of the air-bone gap. Then, due to hearing deterioration in the left ear, a successful stapedotomy was performed in this ear.ConclusionsRevision operations in otosclerosis require a lot of experience of the otosurgeon due to the many reasons for failure, particularly when the first surgery was performed elsewhere. An air-bone gap, severe tinnitus, and balance disorders are indications for reoperation by an experienced otosurgeon, as common causes of failure are postoperative adhesions and subsequent stapes immobilization.

Otologic manifestations of Fanconi anemia and other inherited bone marrow failure syndromes.

The inherited bone marrow failure syndromes (IBMFSs) are diverse disorders with syndrome-specific features; their otologic and audiologic manifestations have not been well described. Our objective was to characterize these in patients with Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond-Blackfan anemia (DBA), and Shwachman-Diamond syndrome (SDS), and to determine the association between physical findings and hearing loss. Patients with an IBMFS underwent comprehensive clinical and laboratory evaluations and testing for syndrome-specific gene mutations. Hearing loss was measured by pure tone audiometry and otologic abnormalities by otomicroscopy. Patients included 33 with FA, 37 with DC, 32 with DBA, and nine with SDS. Hearing loss was most frequent in patients with FA (45%) and DBA (14%). The most common type of hearing loss in FA was conductive (65%). Absent or hypoplastic radius, noted in 21% of the patients with FA, was associated with hearing loss in all cases. Otomicroscopy was abnormal in 66% of patients with FA. Characteristic ear abnormalities included small tympanic membrane (66%), malformed malleus (57%), aberrant tympanic bony island (48%), narrow external auditory canal (EAC) (32%), and abnormal course of chorda tympani (34%). Ear malformations were almost always associated with hearing loss. Hearing loss was rare in patients with DC and SDS. FA is the major IBMFS with associated hearing loss, which is most commonly conductive. Radial hypoplasia or aplasia and characteristic congenital ear malformations are associated with hearing loss in patients with FA. Recognition of these syndrome-specific abnormalities should lead to earlier management of hearing loss.

Transcanal endoscopic ear surgery for pediatric population with a narrow external auditory canal

ObjectivesTo retrospectively determine the size of the external auditory canal (EAC) in a pediatric population and to describe our experience with transcanal endoscopic ear surgery (TEES) in this pediatric population which had been successfully treated for middle ear disease using TEES. MethodsWe analyzed 31 patients ranging in age from 2 to 13 years old (median: 7.6 years) with middle ear disease who underwent TEES between November 2011 and August 2014. Sixteen of these patients had surgery for cholesteatomas; 11 for chronic otitis media; and 4 for malformation of the middle ear. A preoperative CT scan was performed to evaluate the middle ear disease. Transcanal endoscopic tympanoplasty was performed using a rigid endoscope with a 2.7mm outer diameter. Transcanal endoscopic atticoantrotomy was also performed, as necessary, on some patients to access pathologies in the antrum. The values of anterior–posterior diameters and superior–inferior diameters of the bony parts of EAC were measured retrospectively based on the preoperative CT scan data. ResultsTEES was successfully performed in all 31 pediatric patients without resorting to a retroauricular incision. Twenty-seven patients were evaluated for postoperative hearing levels which were found to fall within an acceptable range and for postoperative air-bone gap (ABG) by pure tone audiometry with a resulting mean of 8.6dB. The smallest anterior–posterior diameters of the external ear canal ranged from 3.2 to 7.1mm (5.0±1.0mm) and the smallest superior–inferior diameters ranged from 3.4 to 10.3mm (5.9±1.3mm). ConclusionTEES can be used to safely and effectively treat middle ear disease even in the pediatric population in its narrow EAC.

Prevalence of complications associated with tympanostomy tube insertion

Objective This study aimed to evaluate the prevalence and difficulties during surgery of tympanostomy tube (TT) insertion, complications after surgery, and modalities for management. Background In view of the high incidence of otitis media with effusion in children and the fact that TT is one of its major treatments, we decided to conduct the present observational study. Patients and methods This study included 220 patients with persistent otitis media with effusion after failed medical treatment for 3 months. All patients underwent TT insertion either unilateral or bilateral in 378 operated ears. Results There were 198 problems detected in 378 operated ears. The difficulties during insertion of TT were observed in 82 ears. The most common was the thick mucoid discharge (P = 0.10), followed by the narrow external auditory canals (P = 0.005). Early complications were observed in 42 ears. The most common was early otorrhea (P = 0.03), followed by early extrusion (P Conclusion TT insertion is a temporary procedure performed until the Eustachian tube function returns to its normal function; however, this procedure has many problems during and after insertion. Difficulties of insertion and late side effects are common problems that can be anticipated in nearly one-fifth of the cases. Therefore, ORL surgeons must be aware of the type of difficulty and its management before performing the surgery.

Progressive hearing loss: A case report on surfer's ear

A 30-year-old male reported with the complaint of progressive bilateral hearing loss over a period of six months. There were no associated symptoms of otalgia, tinnitus, discharge or bleeding from the ears. Patient also could not recall any history of trauma to the face. ENT surgeon, who referred the patient for computed tomography (CT) evaluation of hearing loss, had performed the otoscopy and audiometry examinations. Otoscopic findings had revealed, a narrow external auditory canal, tympanic membrane was barely visualized on both sides and was intact. Bilateral moderate conductive hearing loss was noted. He was then advised high resolution computed tomography (HRCT) scan of the temporal bone.

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).

Comparison between endoscopic and microscopic stapes surgery

To investigate whether endoscopic stapes surgery is safer and less invasive than conventional stapes surgery using an operating microscope. Retrospective study. The subjects were 15 patients (15 ears) who underwent endoscopic stapes surgery for otosclerosis or congenital stapedial fixation. Another 35 patients (41 ears) in whom microscopic stapes surgery was performed by the same surgeon were assigned to the control group. The procedures for endoscopic surgery were fundamentally the same as those for microscopic surgery, unless there was no anterior or posterior auricular skin incision. The two surgical techniques were compared with respect to the operating time, postoperative hearing, complications, postoperative pain, and the extent of drilling at the posterosuperior part of the external auditory canal. There were no differences of operating time or postoperative hearing between the endoscopic and microscopic groups. There was very little postoperative pain in the endoscopic group. Postoperative dizziness was mild in all patients who received endoscopic surgery. Drilling at the posterosuperior part of the external auditory canal was less extensive in the endoscopic group than in the microscopic group. Endoscopic surgery is particularly suitable for stapedial disease. Endoscopic stapes surgery can even be done in patients with a curved and narrow external auditory canal. Endoscopic surgery is also suitable for education: The surgical anatomy can be understood easily and both the surgeon and assistants can observe the procedure on the same monitor. However, it should only be performed by experienced surgeons because one-handed manipulation is required and stereoscopic vision is not available.

Endoscopic Myringotomy and Ventilation Tube Insertion under Topical Anesthesia

Endoscopic Myringotomy and Ventilation Tube Insertion under Topical Anesthesia

A family with autosomal dominant oculo-auriculo-vertebral spectrum

Oculo-auriculo-vertebral spectrum (MIM 164210) is a term suggested by Gorlin to summarize the different phenotypic expressions of a continuum that has been known as hemifacial microsomia, Goldenhar syndrome, or first and second branchial arch anomalies. The different terms indicate the extremely variable clinical findings, including especially defects of aural, oral and mandibular development. Additionally, cardiac, renal, skeletal and other anomalies occur. The majority of oculo-auriculo-vertebral spectrum cases are sporadic; nevertheless, several families have been reported with proof of both autosomal dominant and autosomal recessive inheritance. We describe a family with transmission of oculo-auriculo-vertebral spectrum from a mother to her two daughters indicating an autosomal dominant mode of inheritance. Our literature review reveals that patients with autosomal dominant inheritance of oculo-auriculo-vertebral spectrum are more often bilaterally affected than patients with sporadic occurrence of oculo-auriculo-vertebral spectrum. In addition, hearing loss, absent or narrow external auditory canal, anomalies of the mouth and epibulbar dermoids seem to occur less frequently in patients with autosomal dominant oculo-auriculo-vertebral spectrum compared with sporadic oculo-auriculo-vertebral spectrum.

Post-natal short stature, short limbs, brachydactyly, facial abnormalities, and delayed bone age: a new syndrome?

A boy is described with clinical features including post-natal short stature, short limbs, speech delay, relatively large skull, prominent metopic ridge, wide palpebral fissures, proptosis, epicanthic folds, capillary hemangioma between the eyes and nose, flat nasal bridge, everted and small nares, small ears with a narrow external auditory canal and thick lobes, short neck, brachydactyly, bilateral Simian creases, a single flexion crease of the first and fifth fingers, protruding abdomen, and bilateral cryptorchidism. Radiographs did not show any features of bone dysplasia. However, a delayed bone age was noted. The boy's parents are first cousins. To the best of our knowledge, this constellation of anomalies has not been reported before, and may be considered a new syndrome.

Definition of a Critical Region on Chromosome 18 for Congenital Aural Atresia by ArrayCGH

Deletions of the long arm of chromosome 18 occur in approximately 1 in 10,000 live births. Congenital aural atresia (CAA), or narrow external auditory canals, occurs in approximately 66% of all patients who have a terminal deletion 18q. The present report describes a series of 20 patients with CAA, of whom 18 had microscopically visible 18q deletions. The extent and nature of the chromosome-18 deletions were studied in detail by array-based comparative genomic hybridization (arrayCGH). High-resolution chromosome-18 profiles were obtained for all patients, and a critical region of 5 Mb that was deleted in all patients with CAA could be defined on 18q22.3-18q23. Therefore, this region can be considered as a candidate region for aural atresia. The array-based high-resolution copy-number screening enabled a refined cytogenetic diagnosis in 12 patients. Our approach appeared to be applicable to the detection of genetic mosaicisms and, in particular, to a detailed delineation of ring chromosomes. This study clearly demonstrates the power of the arrayCGH technology in high-resolution molecular karyotyping. Deletion and amplification mapping can now be performed at the submicroscopic level and will allow high-throughput definition of genomic regions harboring disease genes.

ダウン症児におけるさん出性中耳炎の経過

In the present study we describe the clinical course and features of otitis media with effusion (OME) in children with Down's syndrome. Of the 190 children with Down's syndrome between aged 3 months to 13 years whom we examined, 94 had unilateral or bilateral OME. We were not able to diagnose the presence or absence of middle ear effusion in 33 children because of narrow external auditory canals or poor patient compliance. We treated and followed 67 children with OME for 1 to 13 years (mean: 3.4 years). Moderate hearing loss was noted in 51% of the children at the first visit. During the follow-up period, resolution of the effusion was observed with age in some cases, and 45 ears were free of effusion. However, only 16 children (24%) showed free of effusion bilaterally. Ten children (13 ears) had sequelae of OME, such as middle ear cholesteatoma, chronic otitis media with perforation and atelectatic ears. Therefore, OME in Down's syndrome appears to be relatively intractable and resistant to therapy. Eighty-eight percent of the 67 children had frequent upper respiratory tract infections, which may contribute to the high incidence and persistence of OME. We conclude that the children with Down's syndrome should be checked otologically and followed from a younger age, and that active treatment is needed for children with moderate hearing loss caused by OME to potentiate their speech and language development.

1209 MESODERMAL INDUCTION DEFECT AS A POSSIBLE CAUSE OF EAR MALFORMATIONS

Deafness due to inner ear anomalies is rarely associated with malformation of the auricles. Two brothers born of consanguineous parents, with profound congenital sensorineural deafness, malformed auricles, abnormal vestibular function and delayed motor development, had low-set, cupped, pointed auricles with folded helices, narrow external auditory canals and normal tympanic membranes. Caloric stimulation failed to elicit nystagmus. Temporal bone laminography revealed bilateral absence of the cochlea and normal ossicles. Since the external and inner ear originate from distinctly separate structures, the embryogenesis of this malformation association is less clear than the more common association of external and middle ear anomalies. In the latter, the first and second branchial arches are the common derivative of both structures. The association of auricular and inner ear anomalies, with sparing of the middle ear, can be explained on the assumption that mesodermal induction is responsible for differentiation of both otocyst and branchial arch ectoderm. A recessive mutant gene may be responsible for the production of a deficient mesodermal inductor substance or a deficient cell surface receptor of the target tissue, rendering it unresponsive. A similar mechanism may be involved in other multiple malformation syndromes, whereby a mutant gene acting during a specific period of organogenesis causes disruption of the normal induction competence relationship.