Perilymph Leakage Research Articles

Clinical course of five patients definitively diagnosed with idiopathic perilymphatic fistula treated with transcanal endoscopic ear surgery.

An idiopathic perilymphatic fistula (PLF) can be difficult to diagnose because patients present with sudden sensorineural hearing loss (SSHL) and/or vestibular symptoms without any preceding events. In such cases, we currently test for cochlin-tomoprotein (CTP) to confirm the diagnosis of idiopathic PLF because CTP is only detected in the perilymph. In this study, we report the clinical course of five patients definitively diagnosed with idiopathic PLF who underwent PLF repair surgery using transcanal endoscopic ear surgery (TEES). Five patients were initially treated with intratympanic dexamethasone for SSHL, at which time a CTP test was also performed (preoperative CTP test). Due to refractory hearing loss and/or fluctuating disequilibrium, PLF repair surgery using TEES was performed to seal the oval and round windows using connective tissue and fibrin glue. These patients were diagnosed with definite idiopathic PLF based on pre- or intra-operative CTP test results (negative, < 0.4 ng/mL; intermediate, 0.4-< 0.8 ng/mL; and positive, > 0.8 ng/mL). We evaluated pre- and intra-operative CTP values, intraoperative surgical findings via a magnified endoscopic view, and pre- and post-operative changes in averaged hearing level and vestibular symptoms. Pre- and intra-operative CTP values were positive and intermediate in three patients, positive and negative in one patient, and negative and positive in one patient. None of the patients had intraoperative findings consistent with a fistula between the inner and middle ears or leakage of perilymph. Only two patients showed a slight postoperative recovery in hearing. Four patients complained of disequilibrium preoperatively, of whom two had resolution of disequilibrium postoperatively. A positive CTP test confirms PLF in patients without obvious intraoperative findings. The CTP test is considered more sensitive than endoscopic fistula confirmation. We consider that CTP test results are important indicators to decide the surgical indication for idiopathic PLF repair surgery. In our experience with the five cases, two of them showed improvements in both hearing and vestibular symptoms.

Prevalence of perilymphatic fistula in patients with sudden-onset sensorineural hearing loss as diagnosed by Cochlin-tomoprotein (CTP) biomarker detection: its association with age, hearing severity, and treatment outcomes

PurposeTo determine the prevalence of perilymphatic fistula (PLF) in sudden-onset sensorineural hearing loss (SSNHL) patients by employing the Cochlin-tomoprotein (CTP) detection test, a specific diagnostic marker for perilymph. We also analyzed the clinical characteristics associated with hearing outcomes in this cohort.MethodsA total of 74 eligible patients were prospectively enrolled. Following myringotomy, middle ear lavage (MEL) samples underwent the CTP test to identify perilymph leakage. Intratympanic dexamethasone (IT-DEX) therapy was administered, and hearing outcomes were assessed. Control groups comprised patients with chronic otitis media (n = 40) and non-inflammatory middle ears (n = 51) with concurrent MEL sample collection.ResultsCTP was positive in 16 (22%) patients. No control samples showed positive results. Multiple regression analysis indicated that age and pre-treatment hearing levels significantly contributed to the CTP value. We found a positive correlation between CTP values, age, and pre-treatment pure-tone averages. Notably, CTP values in SSNHL cases aged 60 and above were significantly higher than in those below 60 years. Patients with positive CTP had significantly worse recovery rates after IT-DEX treatment.ConclusionThis study is the first prospective investigation demonstrating a positive relationship between CTP values, age, and hearing severity in SSNHL, indicating that PLF might be the essential cause of SSNHL, particularly in the elderly. Our findings suggest that IT-DEX may be less effective for PLF-associated SSNHL. Future research could reveal that PLF repair surgery is a viable treatment strategy for SSNHL. This study was registered under the UMIN Clinical Trials Registry (UMIN000010837) on 30/May/2013.

Overall outcome of Modified Radical Mastoidectomy (MRM)

Aims: To compare overall findings of our Modified Radical Mastoidectomy (MRM) practice with the available contemporary standards of international recognition. Design and Methodology: The cohort included the patients who underwent Modified Radical Mastoidectomy from 2013 to 2018 by the author, who were subjected to a retrospective study. The details obtained from clinic reports were preoperative and postoperative hearing (documented to the end of the fourth post op year), and findings that were documented intraoperatively and post operatively in successive clinic visits. The parameters analysed were hearing improvement, disease extension, anatomical variations, immediate and late post-operative complications. Result: There were no evidence of recurrence following the surgical intervention. Postoperative mean air-bone gap had reduced by 9.5dBHL, 5dBHL, 7.44dBHL, 4.9dBHL, 4.1dBHL, 3.75dBHL and 8.18dBHL at 0.125kHz, 0.250kHz, 0.5kHz, 1kHz, 2kHz, 4kHz and 8kHz respectively. Disease involvement was found in Attic, Antrum, Sinus tympani, facial recess, mesotympanum, oval window and posterior sinus, supra tubal recess, sinodural angle cells, sinus sub tympanum, external ear canal, in the descending order of occurrence. 36 audited patients achieved dry ears by the end of second year though some had at least one intermittent wet episode and 4 had continuous persistent wet ears. Intraoperative findings analysis showed 2.5% were observed with perilymph leak, 27.5% had erosion of malleus head by disease, 55% had erosion of incus, 90% intact suprastructure, 97.5% had intact stapes foot plate, 15% had significant dural overhang, 10% had exposed facial nerve, 10% had anterior sigmoid sinus and 2.5% had exposed dura. Immediate post intraoperative complications of 7.5% HB grade 1 facial nerve palsy, 42.5% taste alteration, 5% CSF leak, 25% complaining of disturbing head ache, 7.5% having exposed dura (iatrogenic), 7.5% had dressing removed due to risk of pressure necrosis of pinna or developing Facial nerve palsy and 2.5% (one) patient had the meatus migrating downward due to a connective tissue problem of skin. During post-operative period stitch abscess was noted among 5%, achieved self-cleaning dry ear in 15%, wax build up in the dry ear in 10%. However, over 50% patients had minimum of 1 episode of wet ear in this period. Meatal stenosis and keloid development was noted among 10% and, graft perforation among 20% and 2.5% (one) wound dehiscence. During post-operative period 7.5% developed BBPV and similar percentage had caloric effect during packing of the cavity while 22.5% developed caloric effect during suction of the cavity. Conclusions: The improvements in hearing when adjunct with Tympanoplasty in this audit keep up with recent compared studies. Further its ability to safely achieve a disease free ear is endorsed and complemented by the low complication rates.

A Case of Paroxysmal Positional Vertigo Several Months After Perilymph Fistula Repair

A perilymphatic fistula (PLF) is an abnormal leakage of the perilymph from the inner ear into the middle ear. A PLF may be trauma-induced or may occur with unknown causes. PLF repair should be considered when patients present with sudden or progressive sensorineural hearing loss with positional nystagmus. Although the leakage of perilymph is not always found during the exploration surgery, both hearing loss and dizziness tend to improve immediately after PLF repair. However, about 24% of patients who underwent PLF repair reportedly have experienced recurrent vestibular symptoms, suggesting the possibility of PLF recurrence or other vertigo causes. In this PLF patient, dizziness completely subsided after the PLF repair. However, several months later, the disabling paroxysmal positional vertigo developed, which was diagnosed as benign paroxysmal positional vertigo. Reported herein, with a review of the relevant literature, is a case of paroxysmal positional vertigo several months after PLF repair, where successful vertigo subsidence was finally achieved.

Symptoms: Sudden Hearing Loss and Vertigo

Symptoms: Sudden Hearing Loss and Vertigo

Transcanal Endoscopic Ear Surgery for The Management of Pediatric Perilymphatic Fistula Caused by Trauma

Perilymphatic fistula (PLF) is caused by leakage of perilymph through an abnormal communication between the inner and middle ear. Conservative treatment is considered in the initial stages; however, exploratory tympanotomy is performed if hearing does not improve or if dizziness persists. Transcanal endoscopic ear surgery (TEES) is considered an appropriate treatment option and is gaining popularity. We report a rare case of traumatic PLF in a 7-year-old male patient, in whom pneumolabyrinth without temporal bone fracture was diagnosed and treated by exploratory tympanotomy using TEES, and review the related research to discuss the usefulness of management using TEES for PLF.

Two Cases of Idiopathic Progressive Sensorineural Hearing Loss with Positional Nystagmus in Postpartum Period: Possible Spontaneous Perilymph Fistula?

Perilymph fistula (PLF) is defined as an abnormal communication between the middle ear cavity and perilymphatic space of the inner ear. Most cases of PLF are responsible for pressure change caused by internal trauma or external trauma. However, spontaneous PLF might occur without an obvious history of barotrauma. We report two cases of possible spontaneous PLF in the postpartum period: both cases involved progressive sensorineural hearing loss with positional dizziness. Although the leakage of perilymph was not found during exploration, both hearing loss and dizziness improved immediately after PLF repair. It is necessary to consider the possibility of PLF in patients who have sudden or progressive sensorineural hearing loss with positional nystagmus. Early surgical exploration is recommended even if the history of barotrauma is not clear.

Fetal development and growth of the fissula ante fenestram in the human ear.

Since the fissula ante fenestram (FAF) is considered as a focus of otosclerotic lesion and a route of perilymph leakage, there are few description of prenatal development of the cartilaginous canal passing though the cochlear wall. We examined the sagittal and frontal histological sections of the ear from 32 human fetuses at 8-37 weeks of gestational age. At 8-12 weeks, in the immediately anterior side of a connection between the cochlear and canalicular parts of the otic capsule cartilage, the FAF appeared as a tear of a cartilage between the basal and second turns of the cochlea. The tear became a slit opening to the scala vestibuli. At 13-15 weeks, the FAF, less than 1.2 mm in length, had the anterosuperior and postero-inferior apertures: the former was near the geniculate ganglion and became closed after 15 weeks, while the latter approached the oval window. Third trimester fetuses, the FAF, 1.5-2.0 mm in length, consistently carried a single, postero-inferior aperture extending along the anterior margin of the oval window and it contained no definite epithelium and vessel. Although it was endochondral ossification, there was no clear zonation in cartilage cells of the FAF. A mechanical stress during three-dimensional coiling of the cochlear ducts seemed to provide the FAF. After the FAF was established, the stapes footplate might use a part of the inferior aperture for the syndesmosis. A specific ossification was seen in the FAF, but it might rarely cause the pathological syndesmosis.

Vestibular Terminology and the Cause of Symptoms from a Chronic Perilymph Leak (Perilymph Fistula)

Vestibular Terminology and the Cause of Symptoms from a Chronic Perilymph Leak (Perilymph Fistula)

Endolymphatic Sac Surgery And Posterior Semicircular Canal Fenestration For Meniere's Disease.

Objective: To evaluate the efficiency and safety of the simultaneous endolymphatic sac drainage (ELSD) and posterior semicircular canal fenestration (PSCF) primary on the vestibular function as an new therapeutic strategy in the patients with medically refractory Meniere's disease (MD).Study Design: retrospective follow-up study.Setting: University Clinic of Otolaryngology.Methods: Twenty-six patients with MD with severe vertigo and disability who underwent ELSD and PSCF in the same time in the period of 1988 and 2007 were reviewed. The main outcome measures were frequency of vertigo, functional disability according the guidelines for diagnosis and evaluation of therapy in MD. The canal paresis was evaluated by caloric test. The degree of reduced vestibular response rates as an indicators of the vestibular function were compared before and after surgery.Results: The preoperative audition was already altered in all cases except in 8 patients who had no significant changes in hearing threshold. The mean value of vertigo attacks before operation was 8.6. After 3 years of surgery only one patient (3.8%) had one vertigo attack. Functional level was highly ameliorated except in two patients who presented functional level 2 or B in the late postoperative period. The mean caloric testing duration after 3 years postoperatively showed that the 50% of the patients approaching the normal results.Conclusion: Based on the results of simultaneous endolymphatic sac surgery and posterior canal fenestration, they are effective methods for treatment of the refractory Meniere's disease. Endolymphatic sac surgery enables drainage of endolymphatic fluid and the fenestration of the posterior semicircular canal enables the distension or dilatation of the membranous canal in the decompressed perilymphatic space across the perilymphatic leak at the level of the new fenestra, and, so, appearing of certain decrease of the endolymphatic pressure. Both techniques at the same time decrease the pressure in the case of the endolymphatic hydrops.

Complete Restored Hearing Loss after Excessive Nose Blowing with Various CT Findings

A perilymphatic fistula (PLF) is defined as leakage of perilymph with several possible causes such as superior canal dehiscence through trauma, temporal bone fracture, or sudden pressure change (e.g., skydiving or scuba diving). Pneumolabyrinth can result from temporal bone fracture after trauma, or sudden pressure change in the middle ear or cerebrospinal fluid, such as excessive nose blowing or Valsalva maneuver. A PLF and pneumolabyrinth may occur without trauma, associated with a sudden pressure change in the middle ear. We report two cases of PLF followed by pneumolabyrinth and one case of suspicious PLF without pneumolabyrinth after excessive nose blowing. All three cases were diagnosed as having sensorineural hearing loss and the patients recovered completely after conservative treatment. We report various CT findings of pneumolabyrinth and PLF, from normal CT findings to air pockets in the labyrinth and soft tissue density around the stapes.

Membranous or Hypermobile Stapes Footplate: A New Anatomic Site Resulting in Third Window Syndrome.

Objectives: To describe a potentially underappreciated pathology for post-traumatic persistent intractable dizziness and third window syndrome as well as the methods to diagnose and surgically manage this disorder.Study Design: Observational analytic case studies review at a tertiary care medical center.Methods: Patients suffering persistent dizziness following head trauma and demonstrating Tullio phenomena or Hennebert signs are included. All had reportedly normal otic capsules on high resolution temporal bone CT scans (CT). The gray-scale invert function was used to visualize the stapes footplate, which helped determine the diagnosis. Gray-scale inversion can be used to improve visualization of temporal bone anatomy and pathologic changes when diagnoses are in doubt. A search to check for the presence of perilymph leakage was performed in all cases. This was accomplished using intraoperative Valsalva maneuvers. Fat grafting of round and oval windows was performed.Results: Over an 11-year period between January 2009 and December 2019, 28 patients (33 ears) were treated. Follow-up with balance testing and audiograms were performed 6–8 weeks following surgery. Follow-up ranged from 6 months to 7 years. Prior to surgery all patients reported dizziness in response to loud sounds and/or barometric pressure changes. Seven out of 33 ears had demonstrable perilymph leakage into the middle ear; the rest (26 ears) appeared to have membranous or hypermobile stapes footplates. Membranous stapes footplates were better visualized using the invert function on CT. Thirteen patients had a fistula sign positive bilaterally while 15 had unilateral pathology. Twenty-four of the 28 patients (85.7%) showed both subjective and objective improvement following surgery. No patients suffered from a deterioration in hearing.Conclusions: A previously underappreciated membranous or hypermobile stapes footplate can occur following head trauma and can cause intractable dizziness typical of third window syndrome (TWS). Durable long term success can be achieved by utilizing fat graft patching of the round and oval windows. High resolution temporal bone CT scans using the gray-scale inversion (invert) function can assist in preoperative diagnosis.

Labyrinthine Fistula-Our Experience at a Tertiary Hospital.

This study has aimed to determine the anatomical site of labyrinthine fistula in patients of chronic suppurative otitis media at our centre. Labyrinthine fistulae (LF) are caused by abnormal communications between the inner ear and surrounding structuresresulting in perilymph leakage and hearing loss. Labyrinthine fistula represents as erosive loss of the enchondral bone overlying the semicircular canals without loss of perilymph. The manifestations of fistula like vertigo, hearing loss vary in severity and complexity, commonly ranging from very mild to incapacitating. Cholesteatoma induced fistula most commonly involves lateral semicircular canal probably because of its close proximity to the middle ear, but can involve other semicircular canals and rarely cochlea. This is a retrospective analysis of 36 patients of chronic suppurative otitis media with history of vertigo undergoing tympanomastoid surgery in whom there was an evidence of labyrinthine fistula on HRCT scan of temporal bone. The incidence of patients with labyrinthine fistula presenting with vertigo, nystagmus, sensorineural hearing loss, history of vertigo were analysed. The anatomical location of the fistula was supported by Radiological evidence. Patients underwent either canal wall down mastoidectomy or cortical mastoidectomy. The anatomical site and length of the labyrinthine fistula were analysed. Amongst the 36 patients of chronic suppurative otitis media with labyrinthine fistula 22 (61.1%) patients had atticoantral disease, 4 (11.1%) patients had chronic otitis media with extensive granulation, 2 (5.5%) patients had Tubotympanic disease with polyps, 4 (11.1%) patients had Tuberculous otitis media, 1 (2.77%) patient had Tubotympanic disease with extensive tympanosclerosis eroding the dome of lateral semicircular canal, 1 (2.77%) patient had extensive cholesteatoma with cerebellar abscess, 1 (2.77%) patient had fistula in the promontory following trauma, 1 (2.77%) patient had extensive tympanosclerosis with erosion of promontory. It was noticed that, in 14 (38.88%) patients the fistula was at the centre, in 17 (47.22%) patients the fistula is towards the ampullary end of horizontal semicircular canal and in 5 (13.88%) patients the fistula was towards the non ampullary end of lateral semicircular canal. The maximum length of fistula noticed was 6mm and the minimum length of the fistula noticed was 2mm. Labyrinthine fistula are most commonly noticed in the ampullary end of the lateral semicircular canal. The average length of the fistula was found to be 4mm. Careful elevation of the cholesteatoma matrix over the endosteal membrane and immediate placement of temporal fascia over the exposed fistula is important to avoid injury to the inner ear. Maximum number of fistula were seen in the atticoantral type of Chronic suppurative otitis media. Prior knowledge of anatomical location of the fistulous tract in HRCT temporal bone is important to address the fistula.

Gusher in stapes surgery: a systematic review.

The aim of this study is to perform a systematic literature review on the occurrence of gusher during stapes surgery, to understand its surgical management and outcomes. The PRISMA standard was applied to identify English, Italian or French-language studies, related to stapes surgery and mentioning gusher or perilymphatic leak. Full-texts lacking information on the management of gusher and/or the post-operative hearing outcome were excluded. Twenty-four articles were eventually included. Seventy-six patients were involved in the qualitative synthesis. The management of gusher mostly consisted in covering the oval window and/or filling the tympanic cavity, with absorbable and autologous graft materials. Packing of the external auditory canal was reported in 51 patients (67%). Gusher was related to complete/profound loss of hearing in 25% of the cases and to a worsening of hearing function in 31% of patients. In 19% of patients an improvement in hearing tests was reported; in 28% the hearing function was unchanged. Post-operative vestibular symptoms were reported in 7 patients, and were mainly mild and transient. The absence of vestibular symptoms was underlined in 9 cases, while in 79% of the patients the authors did not provide information. The unexpected occurrence of gusher during stapes surgery represents a relevant issue for the otologic surgeon. Its management most commonly consists in plugging the oval window and the tympanic cavity. In most of the cases, a stapes prosthesis could be positioned. The results on hearing and vestibular functions are widely variable.

Otosclerosis Revisited

Introduction: Otosclerosis usually manifests as a progressive conductive or mixed hearing loss occurring clinically to varying degrees in 0.5% - 1% of the general population. Stapedectomy /stapedotomy is the current treatment of choice for conductive component of Otosclerosis.&#x0D; Materials and Methods: Sixty patients attending the department of ENT of a Medical College Hospital in Bangalore between October 2012 and October 2017 were included in the study.&#x0D; Results: The incidence of otosclerosis is highest in the 3rd and 4th decade. Bilateral symptoms were present in 25% and 75% had unilateral symptoms, right side being more common in our study. Tinnitus and vertigo with deafness are seen in 60% and 25 % of the patients respectively.&#x0D; Ninety percent of the patients presented with moderate to moderately severe hearing loss. Twenty four (40%) out of 60 audiograms studied showed Carhart's notch. Postoperativeclosure of a-b gap to within 10dB could be achieved in 65% cases. The p value calculated comparing pre and post op results is &lt;0.0001 and significant.&#x0D; Vertigo was found to be most common post-operative complication (35%) followed by sensorineural hearing loss (6.6%). Other complications observed during the study include - tympanic membrane tears, foot plate extrusion, lax long process of incus, perilymph leak.&#x0D; Conclusion: From the assessment of the postoperative hearing gain and postoperative complications, it can be concluded that small fenestra stapedotomy gives satisfactory post-operative outcome and may be appropriatein the Indian scenario.

One-Stage Complete Resection of Cholesteatoma With Labyrinthine Fistula: Hearing Changes and Clinical Outcomes.

To evaluate long-term hearing changes and surgical outcomes for middle ear cholesteatoma with labyrinthine fistula following complete surgical removal of the cholesteatoma matrix. Consecutive samples of 43 patients who underwent 1-stage complete resection of the cholesteatoma matrix overlying a labyrinthine fistula were obtained. Immediate and long-term hearing changes were analyzed in association with fistula size. Accuracy of various diagnostic examinations was assessed. Immediately postsurgery, the average bone conduction threshold (43.34 dB) did not differ significantly from the preoperative value (36.66 dB, P = .083). There were also minimal changes thereafter (45.63 dB) without recurrent case over an average follow-up time of 38.3 months (range, 17-69 months). More than 10 dB hearing loss was found in 7 patients with a case of intraoperative perilymph leakage (2.3%), although 5 of them had had preoperative air conduction threshold above 90 dB. Their fistulas were significantly larger than those of patients without hearing loss ( P = .027). Although caution is required for total removal of a large fistula, owing to increased risk of postoperative hearing deterioration, 1-stage complete resection of cholesteatoma matrix on labyrinthine fistula could be effective in disease control and long-term hearing preservation.

The diagnostic performance of a novel ELISA for human CTP (Cochlin-tomoprotein) to detect perilymph leakage.

Perilymphatic fistula is defined as an abnormal communication between the perilymph-filled space and the middle ear, or cranial spaces. The manifestations include a broad spectrum of neuro-otological symptoms such as hearing loss, vertigo/dizziness, disequilibrium, aural fullness, tinnitus, and cognitive dysfunction. By sealing the fistula, perilymphatic fistula is a surgically correctable disease. Also, appropriate recognition and treatment of perilymphatic fistula can improve a patient’s condition and hence the quality of life. However, the difficulty in making a definitive diagnosis due to the lack of an appropriate biomarker to detect perilymph leakage has caused a long-standing debate regarding its management. We have reported a clinical test for the diagnosis of perilymphatic fistula by detecting a perilymph specific protein, Cochlin-tomoprotein, as a diagnostic marker using a western blot. The aim of this study is to establish an ELISA-based human Cochlin-tomoprotein detection test and to evaluate its diagnostic accuracy in clinical subjects. The results of ELISA showed good dilution reproducibility. The mean concentration was 49.7±9.4 of 10 perilymph samples. The ROC curve in differentiating the perilymph leakage condition from the normal middle ear was significant (P < 0.001) with an area under the curve (AUC) of 0.918 (95% CI 0.824–0.100). We defined the diagnostic criteria as follows: CTP<0.4 negative; 0.4≦CTP<0.8 intermediate; 0.8≦CTP(ng/ml) positive in the clinical usage of the hCTP ELISA, and sensitivity and specificity were 86.4% and 100%, respectively. We further tested the expression specificity of the Cochlin-tomoprotein by testing blood and CSF samples. The concentration was below the detection limit (0.2 ng/ml) in 38 of the 40 blood, and 14 of the 19 CSF samples. We report the accuracy of this test for the diagnosis of perilymphatic fistula. Using ELISA, we can improve the throughput of the test. Furthermore, it is useful for a large-scale study to characterize the clinical picture and delineate the management of this medical condition.

Hearing Preservation During Cochlear Implantation and Electroacoustic Stimulation in Patients With SLC26A4 Mutations

Patients with SCL26A4 mutations presenting with Mondini deformity and enlarged vestibular aqueduct (EVA) tend to have comparable residual hearing. Although cochlear implantation (CI) produces good results in this group, deterioration of residual hearing can be an adverse event after surgery due to accompanying cochlear malformation and perilymph leakage during cochleostomy. The purpose of this study was to investigate if CI in patients with SCL26A4 mutations via the round window (RW) approach could achieve preservation of residual hearing, and to evaluate their speech reception with electroacoustic stimulation (EAS). This is a retrospective chart review of eight patients with bilateral EVA, who were bi-allelic patients with SCL26A4 mutations. CI was performed in all patients by a single surgeon using the RW approach. Audiological results were compared before and after implantation. Additional hearing loss after CI was less than 10 dBHL in five out of eight patients. Average hearing deterioration after CI was 8.75 dB (range, 0-26). Six out of eight patients used EAS mode after CI. The acoustic stimulation frequency ranged from 271 to 438 Hz. Patients showed better speech recognition in quiet and in noise using EAS mode compared with electrical stimulation alone. Preservation of residual hearing could be achieved after CI in patients with the SLC26A4 mutation via the RW approach. For successful preservation of residual hearing, application of newly-developed soft electrode and meticulous surgical is necessary. Our study showed that patients with the SLC26A4 mutation can be good candidates for EAS surgery.

Diagnostic criteria of barotraumatic perilymph fistula based on clinical manifestations

Conclusions: Patients with sudden hearing loss and dizziness after barotrauma should be suspected of barotraumatic perilymph fistula (PLF). Early surgical repair of PLF showed better hearing outcomes. Therefore, diagnostic criteria should help surgical indications of barotraumatic PLF.Objectives: The aim of this study was to establish diagnostic criteria for barotraumatic PLF.Methods: Twenty-four patients (26 ears) underwent surgery on suspicion of barotraumatic PLF. The causes of barotrauma and clinical symptoms were analyzed by surgical findings. Diagnostic criteria of PLF were proposed according to its clinical manifestations.Results: Definite PLF (17 subjects) was confirmed by any evidence of perilymph leak through oval and round windows. For the other seven subjects (probable PLF), even though there was no evidence of perilymph leak, their clinical manifestations were similar to definite PLF. High frequency hearing loss occurred as an early symptom after barotrauma. Positional dizziness occurred a few hours after auditory symptoms (67%). Positional nystagmus was observed in 10 cases. The characteristics of positional nystagmus were multi-directional, longer in duration, smaller in amplitude, no reversibility, and no response to repositioning maneuver. Regarding the surgical outcomes, hearing was significantly improved in the early repaired PFL group, and dizziness was improved in 96% of patients.

A Case of Perilymphatic Fistula Misdiagnosed as Benign Paroxysmal Positional Vertigo

Perilymphatic fistula (PLF) is an uncommon disease characterized by abnormal leakage of the perilymph from the inner ear into the middle ear. The symptoms of PLF include sudden-onset hearing loss, tinnitus, ear fullness, and vertigo resulting from inner-ear trauma, stapedial surgery or barotrauma such as valsalva, and nose blowing. As nystagmus can be induced by the affected-ear in down position, benign positional paroxysmal vertigo (BPPV) should be considered for differential diagnosis. About 40-50% of PLF patients have experienced spontaneous healing, but surgical repair should be considered when the hearing symptom and dizziness are not alleviated or are aggravated within a few days. Reported herein, with a review of the relevant literature, is a case of PLF initially misdiagnosed as BPPV but where successful fistula repair was finally achieved.