Novel GNAI3 mutation in a Chinese family with auriculocondylar syndrome and treatment of severe dentofacial deformities: a 5-year follow-up case report.

BACKGROUND: Auriculocondylar syndrome (ARCND) is an extremely rare autosomal dominant or recessive condition that typically manifests as question mark ears (QMEs), mandibular condyle hypoplasia, and micrognathia. Severe dental and maxillofacial malformations present considerable challenges in patients' lives and clinical treatment. Currently, only a few ARCND cases have been reported worldwide, but most of them are related to genetic mutations, clinical symptoms, and ear correction; there are few reports concerning the treatment of dentofacial deformities. CASE PRESENTATION: Here, we report a rare case of ARCND in a Chinese family. A novel insertional mutation in the guanine nucleotide-binding protein alpha-inhibiting activity polypeptide 3 (GNAI3) was identified in the patient and their brother using whole-exome sequencing. After a multidisciplinary consultation and examination, sequential orthodontic treatment and craniofacial surgery, including distraction osteogenesis and orthognathic surgery, were performed using three-dimensional (3D) digital technology to treat the patient's dentofacial deformity. A good prognosis was achieved at the 5-year follow-up, and the patient returned to normal life. CONCLUSIONS: ARCND is a monogenic and rare condition that can be diagnosed based on its clinical triad of core features. Molecular diagnosis plays a crucial role in the diagnosis of patients with inconspicuous clinical features. We present a novel insertion variation in GNAI3, which was identified in exon 2 of chromosome 110116384 in a Chinese family. Sequential therapy with preoperative orthodontic treatment combined with distraction osteogenesis and orthognathic surgery guided by 3D digital technology may be a practical and effective method for treating ARCND.

A Novel Method for Patients With Ear Keloid: Enucleation and Fat Grafting.

BACKGROUND: Keloid is a dermal fibroproliferative disease unique to humans. Due to the ambiguity in its pathophysiology and the frequent recurrence of keloid, there is no clear consensus on the treatment of keloid and there are many treatment methods defined. In order to benefit from the positive effects of fat grafting on pathological scars, we applied fat grafting to patients who underwent keloid enucleation. METHODS: Fifteen ear keloid patients included in the study. All patients underwent the same surgical procedure by the same surgeon. Routine follow-ups and examinations were performed to evaluate the results and in addition, the Patient and Observer Scar Assessment Scale (POSAS) survey was used. RESULTS: In the study, 15 patients were followed for a median (IQR) period of 21 (13-28) months. No recurrence was observed in any patient during follow-up, which occurred for a median of 21 (13-28) months. In the questionnaire filled out by the patients, the preoperative median value was found to be 48 (IQR: 12), whereas the postoperative median value was found to be 14 (IQR: 8). According to the patients, there was a statistically significant ( P < 0.05) positive improvement after surgery. CONCLUSIONS: Historically, surgical procedures were avoided because the surgical recurrence rate was very high, but today, recurrence rates are decreasing with combined treatments. These treatment combinations may require more than one intervention and require frequent clinical follow-ups. With our technique of fat grafting after enucleation, the treatment was completed with a single operation and no additional intervention was required.

Variant Types of Preauricular Sinuses: Classifications, Clinical Presentation and Management.

OBJECTIVE: To make surgeons aware of the differing types of preauricular sinuses (PAS), we summarize our experience with diagnosis and treatment of varying types of PAS. METHODS: We retrospectively reviewed clinical data from patients who had undergone preauricular fistulectomy between March 2015 and March 2020. These patients were categorized into two groups according to locations of congenital fistula pit. RESULTS: Twelve patients with variant PAS accounted for 6.8% (12/177) of all patients. The variant types of PAS could be classified into three types (from type 1 to type 3), based on the location of the fistula pit. Type 1 (seven patients; eight ears) patients had pits located on the ascending helix crus, whereas type 2 (four patients, four ears) and type 3 (one patient, one ear) patients had pits located on the external auditory canal (EAC) and lobule, respectively. Fistular tracts penetrated the cartilage of the helix crus in seven of the type 1 variant ears. Swelling and discharge were located at the ascending helix crus (in four ears), cavum concha (in two ears), and posterior to the auricle (in one ear). In four of the type 2 ears, the fistular tracts were located at the anterior margin of the ascending limb of the helix. CONCLUSION: Fistula tracts where fistula pit occurred on the ascending helix crus were more likely to penetrates through the cartilage, and fistula tracts with fistula pits that occurred on the EAC were adjacent to the cartilage of the ascending helix and tragus. Meticulous dissection and complete removal of fistula tissue are critical to avoid postoperative recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:3839-3845, 2024.

Safety, tolerability, and effect of a single aural dose of Dornase alfa at the time of ventilation tube surgery for otitis media: A Phase 1b double randomized control trial.

BACKGROUND: One third of children require repeat ventilation tube insertion (VTI) for otitis media. Disease recurrence is associated with persistent middle ear bacterial biofilms. With demonstration that Dornase alfa (a DNase) disrupts middle ear effusion biofilms ex vivo, we identified potential for this as an anti-biofilm therapy to prevent repeat VTI. First, safety and tolerability needed to be measured. METHODS: This was a phase 1B double-blinded randomized control trial conducted in Western Australia. Children between 6 months and 5 years undergoing VTI for bilateral middle ear effusion were recruited between 2012 and 2014 and followed for two years. Children's ears were randomized to receive either Dornase alfa (1 mg/mL) or 0.9 % sodium chloride (placebo) at time of surgery. Children were followed up at 2 weeks post-VTI and at 3-monthly intervals for 2 years. Outcomes assessed were: 1) safety and tolerability, 2) otorrhoea frequency, 3) blocked or extruded ventilation tube (VT) frequency, 4) time to blockage or extrusion, 5) time to infection recurrence and/or need for repeat VTI. RESULTS: Sixty children (mean age 2.3 years) were enrolled with 87 % reaching study endpoint. Treatment did not change otorrhoea frequency. Hearing improved in all children following VTI, with no indication of ototoxicity. Dornase alfa had some effect on increasing time until VT extrusion (p = 0.099); and blockage and/or extrusion (p = 0.122). Frequency of recurrence and time until recurrence were similar. Fourteen children required repeat VTI within the follow-up period. CONCLUSION: A single application of Dornase alfa into the middle ear at time of VTI was safe, non-ototoxic, and well-tolerated. TRIAL REGISTRATION: ACTRN12623000504617.

Registration of preoperative temporal bone CT-scan to otoendoscopic video for augmented-reality based on convolutional neural networks.

PURPOSE: Patient-to-image registration is a preliminary step required in surgical navigation based on preoperative images. Human intervention and fiducial markers hamper this task as they are time-consuming and introduce potential errors. We aimed to develop a fully automatic 2D registration system for augmented reality in ear surgery. METHODS: CT-scans and corresponding oto-endoscopic videos were collected from 41 patients (58 ears) undergoing ear examination (vestibular schwannoma before surgery, profound hearing loss requiring cochlear implant, suspicion of perilymphatic fistula, contralateral ears in cases of unilateral chronic otitis media). Two to four images were selected from each case. For the training phase, data from patients (75% of the dataset) and 11 cadaveric specimens were used. Tympanic membranes and malleus handles were contoured on both video images and CT-scans by expert surgeons. The algorithm used a U-Net network for detecting the contours of the tympanic membrane and the malleus on both preoperative CT-scans and endoscopic video frames. Then, contours were processed and registered through an iterative closest point algorithm. Validation was performed on 4 cases and testing on 6 cases. Registration error was measured by overlaying both images and measuring the average and Hausdorff distances. RESULTS: The proposed registration method yielded a precision compatible with ear surgery with a 2D mean overlay error of 0.65 ± 0.60 mm for the incus and 0.48 ± 0.32 mm for the round window. The average Hausdorff distance for these 2 targets was 0.98 ± 0.60 mm and 0.78 ± 0.34 mm respectively. An outlier case with higher errors (2.3 mm and 1.5 mm average Hausdorff distance for incus and round window respectively) was observed in relation to a high discrepancy between the projection angle of the reconstructed CT-scan and the video image. The maximum duration for the overall process was 18 s. CONCLUSIONS: A fully automatic 2D registration method based on a convolutional neural network and applied to ear surgery was developed. The method did not rely on any external fiducial markers nor human intervention for landmark recognition. The method was fast and its precision was compatible with ear surgery.

Eustachian tube balloon dilation.

Eustachian tube dysfunction is a frequent pathology of middle-ear ventilation, leading to many more or less disabling otologic symptoms. Severity varies from simple otitis media with effusion to aggressive chronic otitis or cholesteatoma. First-line treatment is medical, but surgery can also be proposed. It consists in balloon dilation of the Eustachian tube, in order to increase ventilation and improve symptoms. It is performed in second-line in patients with Eustachian tube dysfunction confirmed by tubomanometry, after failure of local drug treatment. It can be performed under general or local anesthesia but it is mandatory to use a medical device with market authorization for this indication. The main aim of this surgical note is to describe the technique of balloon dilation in the treatment of Eustachian tube dysfunction and the three balloon models with CE label and market authorization currently available in France and in Europe. Balloon dilation of the Eustachian tube appears to be a promising option to restore natural middle-ear ventilation in patients with chronic obstructive Eustachian tube dysfunction.

Treatment of auricular pseudocysts using enhanced negative drainage: a prospective study of 21 cases.

OBJECTIVE: Auricular pseudocysts are rare, painless, benign intracartilaginous cysts of the auricle that are not lined by epithelium and have no known aetiology. METHOD: This was a prospective study conducted in an ENT department from January 2020 to June 2022. In 21 patients, complete aspiration of the pseudocyst with enhanced negative drainage was performed. They were followed for a minimum of six months. RESULTS: All patients completely responded to the negative drainage treatment. No cases of recurrence or obvious deformities were observed. CONCLUSION: Aspiration with intensified negative drainage was associated with a positive response in patients with auricular pseudocysts. Complete resolution of the swelling can be achieved without any serious complications. Thus, it appears to be a simple and effective method for managing the condition.

Balloon dilation improves both the hearing level and the quality of life in patients suffering from obstructive Eustachian tube dysfunction.

PURPOSE: Chronic obstructive Eustachian tube dysfunction (OETD) can lead to tympanic membrane (TM) retraction and middle ear effusion (MEE) which can cause conductive hearing impairment, which among other ear symptoms can lower the quality of life (QoL). In this prospective study we assess hearing results and subjective changes in QoL following balloon Eustachian tuboplasty (BET) in treatment of OETD. METHODS: Totally 25 ears with TM retraction and 18 ears with MEE due to chronic OETD underwent BET as the sole intervention. Outcomes including otoscopy, ability to perform the Valsalva maneuver, tympanometry, audiometry, Eustachian tube inflammation scale and the Glasgow Benefit Inventory questionnaire (GBI) were obtained on all patients preoperatively and 6 months postoperatively. RESULTS: Hearing thresholds improved statistically significantly (p < 0.05) with means of 3 dB in the TM retraction group and 9 dB in the MEE group. Total GBI results indicated a positive influence on patients' QoL. Valsalva success rate was 80% in patients with TM retraction and 67% in patients with MEE. Tympanometry results improved in 50% of TM retraction patients and in 33% of MEE patients. CONCLUSIONS: Here we demonstrated that the BET has a positive impact on patients' conductive hearing loss and QoL in patients with TM retraction or MEE. Results were better in TM retraction group than in MEE group.

Balloon Eustachian Tuboplasty-A Feasible Double-Blinded Sham Surgery Randomized Clinical Trial Protocol to Study Efficacy.

INTRODUCTION: Balloon Eustachian tuboplasty (BET) is used to treat obstructive Eustachian tube dysfunction (OETD) and recurrent otitis media with effusion (OME). However, there are no indisputable evidence of its efficacy. Here, we present a multicenter, double-blinded, randomized, placebo-controlled trial (MDRCT) design to evaluate the efficacy of BET, and the results of a pilot trial with 3- and 12-months' follow-up. MATERIAL AND METHODS: This was a prospective MDRCT. For a pilot study, OETD (n = 10) and OME (n = 5) patients were recruited and followed. Detailed inclusion and exclusion criteria were used. Participants were randomized at beginning of the operation to active or sham surgery. All procedures were performed under local anesthesia. Controls were performed in double-blinded manner (both patient and physician), at 3 and 12 months after the procedure. RESULTS: Altogether, 20 ears were treated and followed for 12 months, including 14 active BETs and 6 sham surgeries. Both the active and sham surgery were performed under local anesthesia without problems or deviations from the protocol. There were no differences in the preoperative symptoms (ETDQ-7) or objective measures (tympanometry, Valsalva and Toynbee maneuvers, tubomanometry, Eustachian tube score) between active and sham surgery arms. During follow-up, we noticed largely similar reduction in subjective symptoms and improvement in Eustachian tube score both in active and sham surgery arms. CONCLUSIONS: The pilot study demonstrates that our MDRCT protocol is feasible, and that blinded RCTs are dearly needed to objectively measure the efficacy of BET. LEVEL OF EVIDENCE: 2 Laryngoscope, 134:1874-1881, 2024.

Endoscopic approach to geniculate ganglion: a multicentric experience.

PURPOSE: A variety of lesions could arise from the GG area, or extend into this region from adjacent sites. The management of perigeniculate lesions includes observation, surgery, and radiation, according to the nature, the size of the lesion, and the accompanying symptoms. Preliminary experiences on the exclusive transcanal endoscopic approach to the GG area have shown safety and feasibility avoiding of any postauricular incision, or brain manipulation. The experience from two referral centers on patients treated for a GG lesion with a totally endoscopic approach is herein reported. METHODS: Data about patients who underwent exclusive endoscopic approach to the GG area at the Otolaryngology Departments of the University Hospitals of Modena and Bologna between May 2017 and February 2022 were retrospectively collected. RESULTS: The total number of patients included in our study was 11. 10 patients (91%) had progressive unilateral facial paralysis and 1 patient (11%) presented with chronic otorrhea. The mean largest diameter of the treated lesions was of 8 mm. The resection was extended to the fundus of the IAC in 2 patients (expanded approach). The remaining 9 patients (82%) underwent partial ossicular replacement prosthesis (PORP). No major complications occurred. Facial nerve outcomes were good in all patients and the mean ABG worsened from 12 dB pre-operatively to 22 dB post-operatively. CONCLUSIONS: The exclusively endoscopic approach to GG lesions represents a viable alternative to traditional microscopic approaches and may be included in the armamentarium of ear surgeons.

Permanent occlusion of the Eustachian tube: a retrospective study on reopening procedures.

PURPOSE: This study retrospectively evaluated the efficacy and versatility of reopening procedures for the permanent occlusion of the cartilaginous Eustachian tube (POET) by analyzing four consecutive cases. METHODS: The study included all patients diagnosed with POET who suffered from Eustachian tube occlusion and glue ear. A combined approach of endoscopic transnasal/transoral laser surgery was utilized to reopen the POET. This was subsequently followed by balloon dilation (BET) and stenting for a duration of six weeks. In one distinct case, the Eustachian tube orifice was approached via a transtympanic method, where a balloon catheter was placed. The primary outcome measures targeted the success rate of reopening, which was quantified using audiological outcomes and Eustachian tube patency verified by a positive Valsalva maneuver. RESULTS: Four patients, with an age range of 14-62 years (mean age of 29.3 years), were subject to Eustachian tube reopening. The duration of follow-up varied between 10 and 24 months, averaging at 16.2 months. Notably, 75% of the surgically treated ears displayed no evidence of glue ear upon their last follow-up and showed restoration of Eustachian tube patency. The procedures were executed without any surgical complications. The causes for POET in these patients were heterogeneous: two were attributed to scarring post adenoidectomy, one to occlusion following orthognathic surgery and the remaining one due to prior radiotherapy treatment for squamous cell carcinoma located at the soft palate. DISCUSSION: Total occlusion of the cartilaginous Eustachian tube may be linked to persistent middle ear diseases. It is imperative to conduct nasopharyngeal endoscopy in these cases. The findings from this study suggest that the Eustachian tube reopening procedure is predominantly effective and safe for patients with POET stemming from a variety of pathologies. Future research should focus on exploring advanced stenting devices and necessitate longer follow-up periods for comprehensive understanding.

Mid-term evaluation of the surgical management of patulous Eustachian tube dysfunction: a STROBE observational study.

INTRODUCTION: Patulous Eustachian tube (PET) is a condition affecting approximately 0.3% to 6.6% of the population, with autophony being the predominant complain. The management of PET lacks a well-defined standard in the literature as no effective medical treatments have been documented but various surgical options are available. This study aims to report mid-term outcomes following surgical management of PET. MATERIALS AND METHODS: All patients who underwent surgical intervention for PET between September 2017 and June 2022 were enrolled. Data encompassing general demographics, quality of life (GBI), and procedure-specific data were collected. RESULTS: A total of 30 PET cases (in 19 patients) underwent surgical intervention including 9 injections of hyaluronic acid, 13 fat injections, 6 endoscopic shim insertions, 1 cartilage graft, and 1 injection of hydroxy apatite. After an average follow-up of 22 ± 14 months, 16 cases (53%) achieved complete symptom relief, while 8 cases (26.6%) reported partial relief. Additionally, 11(36%) cases required multiple surgeries. No specific surgical technique demonstrated superiority. Quality of life improved in 77% of cases based on 10 out of 13 GBI collected. Recurrence of PET symptoms occurred on average 10.6 ± 9.7 months after initial surgery, with an estimated global risk of 75% at 3 years. Transient serous otitis media was observed in only 4 cases (13.3%). CONCLUSION: Surgical intervention for PET was found to be effective, achieving complete symptom relief in 53% of cases and significantly improving quality of life 2 years post-surgery. However, a substantial portion of cases necessitated one or more re-interventions. The durability of effectiveness appears to diminish over time.

Attic reconstruction techniques in cholesteatoma surgery: a comparative analysis of cartilage versus bone pate.

BACKGROUND: Cholesteatoma surgery involves canal wall down (CWD) and canal wall up (CWU) mastoidectomy. CWU is associated with higher cholesteatoma recurrence, often linked to attic retraction pockets. Attic reconstruction with cartilage or bone pate lacks comparative evidence. AIMS/OBJECTIVES: To compare the effectiveness of cartilage and bone pate in attic reconstruction during CWU mastoidectomy for cholesteatoma. MATERIAL AND METHODS: We conducted a retrospective study at King Abdullah University Hospital (KAUH) in Jordan, analyzing surgeries performed from 2011 to 2021. Patients who underwent CWU mastoidectomy with attic reconstruction using tragal cartilage with perichondrium or bone pate were included. RESULTS: Of 48 patients analyzed, 26 had cartilage graft attic reconstruction, and 22 received bone pate. Recurrent cholesteatoma occurred in 19.23% of the cartilage group but none in the bone pate group (p = .001). Ear discharge was observed in 19.23% of the cartilage group and 18.18% of the bone pate group, while tympanic membrane perforations and external auditory canal cholesteatoma were more prevalent in the cartilage group. CONCLUSIONS AND SIGNIFICANCE: Our study indicates that bone pate results in significantly lower cholesteatoma recurrence than cartilage grafting in CWU mastoidectomy attic reconstruction. Bone pate offers stability and favorable long-term outcomes for outer attic wall repair.

[Contemporary diagnosis and management of congenital microtia and aural atresia : Part 1: Principles and diagnosis]. / Aktuelle Diagnostik und Therapie bei Ohrmuscheldysplasien und Gehörgangsfehlbildungen : Teil 1: Grundlagen und Diagnostik.

Congenital malformations of the pinna and aural atresia can result in major aesthetic and functional deficits. Knowledge about embryologic developments and established classification systems is an essential requirement when dealing with affected patients. Early detection of deficiencies and introduction of appropriate diagnostic measures is vital to initiate adequate therapies and prevent long-term disabilities. Treatment for malformations of the pinna-if requested-is mostly surgical, infrequently an epithesis is applied. As in other surgical fields, tissue engineering will likely play a crucial role in the future. Treatment of aural stenosis and atresia aims at improvement of hearing levels and prevention of secondary complications like cholesteatoma and chronic otorrhea. Auditory rehabilitation comprises a spectrum from conventional hearing aids to invasive hearing implants, the latter being favored in recent years.

Impact of palatoplasty techniques on tympanic membrane findings and hearing prognosis in children with cleft palate.

OBJECTIVE: Children with cleft palate (CP) are at high risk of developing otitis media with effusion (OME) due to Eustachian tube (ET) dysfunction. Palatoplasty has been reported to decrease the frequency of middle ear disease and improve ET function, and although various techniques have been developed, there is no consensus on the differences in the impact of different techniques on the middle ear. The purpose of this study was to determine the differential effects of palatoplasty on middle ear function and hearing. METHODS: We performed a retrospective observational survey of pediatric patients who underwent palatoplasty for CP between June 2010 and October 2018 at Tohoku University Hospital. Cases were divided into three groups depending on the palatoplasty procedures performed: the push-back palatoplasty group, the two-flap palatoplasty group, and the Furlow double-opposing Z-plasty group. We examined the differences in clinical characteristics between patients who underwent each procedure. The primary outcome variable was tympanic membrane (TM) findings, and the secondary outcome was hearing test results. RESULTS: Children who underwent the two-flap palatoplasty had a higher tympanostomy tube (TT) insertion rate and a higher total number of TT insertions than those who underwent the Furlow double-opposing Z-plasty or the push-back palatoplasty. The TM retraction rate tended to be lower in the Furlow double-opposing Z-plasty group than in the push-back palatoplasty group or the two-flap palatoplasty group. The hearing test results at the last visit were not significantly different among the three groups. CONCLUSIONS: Children who underwent the two-flap palatoplasty had a higher rate of TT insertions, potentially increasing the risk of TM perforation. In contrast, the Furlow double-opposing Z-plasty group had a lower tendency for TM regression, possibly due to improved ET function and reduced incidence of OME. It is important to understand the advantages and disadvantages of each technique before selecting one suitable for the child's cleft and arch width. Additionally, it is important to conduct regular follow-up of TM findings and hearing test results even after palatoplasty.

Autologous Fat Injection for the Treatment of Patulous Eustachian Tube.

BACKGROUND: Patulous eustachian tube is a disorder that leads to disturbing symptoms such as autophony, respiratory noise, and aural fullness. There has not been an established treatment found for this disorder. This study aims to assess the efficacy of autologous fat injection for a novel treatment of patients with patulous eustachian tube. METHODS: Twenty-eight patients with refractory patulous eustachian tube were recruited for the study. Endoscopic autologous fat injection was performed submucosally into the anterior and posterior portion of the pharyngeal orifice of the eustachian tube. The outcomes were quantitatively assessed by comparing the difference between pre- and postoperative scores with the visual analog scale on 4 criteria: aural fullness, respiratory noise, tinnitus, and autophony. RESULTS: Autologous fat injection was performed successfully in all patients without major complications. According to the combined visual analog scale scores, after the treatments, 20 patients showed significant improvement and 6 showed moderate improvement. The overall success rate of the treatment was 92.9%. CONCLUSION: Autologous fat injection is an effective and safe procedure for the treatment of patulous eustachian tube.

Outcomes of Spontaneous Cerebrospinal Fluid Leak Repair With Concurrent Eustachian Tube Dysfunction.

OBJECTIVE: To compare the presentation and outcomes of patients with and without obstructive eustachian tube dysfunction (oETD) undergoing repair of lateral skull base spontaneous cerebrospinal fluid (sCSF) leaks. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Adults with lateral skull base sCSF leaks who underwent repairs from January 1, 2011, to December 31, 2020, were collected. MAIN OUTCOME MEASURE: Comparative statistics and effect sizes were used to compare clinical features, operative findings, and outcomes between groups. RESULTS: Of 92 ears from 89 patients included, 51.1% (n = 47) had oETD. There were no differences in demographics between patients with and without oETD. Mean age was 60.7 ± 13.1 versus 58.5 ± 12.8 years ( d = -0.17 [-0.58 to 0.24]), mean body mass index was 33.8 ± 8.5 versus 36.0 ± 8.0 kg/m 2 ( d = 0.27 [-0.14 to 0.68]), and female sex preponderance was 59.6% (n = 28) versus 68.8% (n = 31; Φ = -0.09), respectively. There were no differences in the radiologic number, size, and locations of defects. Patients with oETD had less pneumatized mastoids than those without oETD ( p = 0.001; Φ = 0.43). Mean change from preoperative to postoperative air pure-tone average for those with and without oETD was -1.1 ± 12.6 versus 0.1 ± 17.2 dB ( d = 0.09 [-0.04 to 0.58]), respectively. Six ears (6.5%; three with and three without oETD) underwent revisions for rhinorrhea/otorrhea between 5 and 28 months postoperatively, during which four leaks were found, the two patients without leaks had oETD. CONCLUSIONS: The presentation of sCSF leaks and outcomes of repairs in patients with oETD do not differ from those without oETD. Although postoperative otorrhea might represent an inflammatory or infectious process in patients with oETD, reexploration is warranted if patients do not improve with conservative treatment.

Acquired Stenosis of the External Ear Canal.

Acquired stenosis of the external ear canal (ASEEC) is a relatively uncommon condition. Stenosis or narrowing of the external ear canal (EEC) occurs lateral to the tympanic membrane resulting in a skin lined blind canal. Recurrent otorrhea, and conductive hearing loss are typical clinical features. Although ASEEC can be due to different etiologies, a common pathogenesis, namely an inflammatory cascade, has been implicated. Clinical evaluation, audiogram, and Computed tomography (CT scan) form the mainstay of diagnosis. Surgery is the primary modality for treatment. Restenosis is the most common postsurgical complication.

External Ear Disease: Keratinaceous Lesions of the External Auditory Canal.

Keratosis obturans (KO) and external auditory canal cholesteatoma (EACC) are two distinct keratinaceous lesions of the external ear. This article reviews the signs, symptoms, pathophysiology, workup, and treatment of each. Patients with either pathology can often be managed in the clinic with debridement; however, EACC is more likely to involve osteonecrosis and require more extensive operative management if disease is not confined to the canal on account of the bony erosion characteristic of cholesteatoma. If required for extensive disease, surgical approaches to both pathologies are similar.

Modified-modified radical mastoidectomy.

PURPOSE: It is unusual to have communication from the external auditory canal (EAC) directly to the mastoid, totally sparing the tympanum. These patients need a different surgical approach, a modified canal wall-down procedure, to completely clear the disease but fully preserve the tympanum. We present one such exceptional case. CASE PRESENTATION: A 28-year-old lady presented with ear discharge for 1 year. Imaging confirmed the canal-mastoid fistula, but the entire tympanum was normal. We performed a modified-modified radical mastoidectomy. CONCLUSIONS: Canal-mastoid fistula is an infrequent entity and may be idiopathic. Despite being evident on clinical examination, imaging aids in assessing size and location of the defect. Although EAC reconstruction may be attempted, the majority require a canal wall-down procedure.