Perilymphatic Fistula Research Articles

Cholesteatoma Induced Labyrinthine Fistula: Is Aggressiveness in Removing Disease Justified?

Issues of complete disease clearance and hearing preservation in cholesteatoma induced labyrinthine fistula cases has been discussed and updated in this article. Successful disease clearance and hearing preservation in a case of cholesteatoma induced isolated cochlear promontory fistula encouraged us to retrospectively analyse 13 more cases of cholesteatoma induced labyrinthine fistula who presented in emergency service as complicated chronic suppurtive otitis media. Pre-operatively nine patients experienced vertigo, two had profound sensori neural hearing loss and radiology was suggestive of labyrinthine fistula in 12 patients. Lateral semicircular canal was involved in 13 cases. In all cases cholesteatoma matrix was completely removed from the fistula site irrespective of the fistula size and hearing status. Hearing was preserved in 11 out of 12 patients. Gentle and meticulous removal of the matrix and careful repair of labyrinthine fistula delivers significant hearing preservation rate along with a safe and dry ear which avoids a second look surgery.

Endoscopic management of congenital cholesteatoma

Congenital cholesteatomas are epithelial cell rests found within the middle ear or mastoid that are present behind an intact tympanic membrane. Typically found early in life, these expansile skin cysts can result in conductive hearing loss by impeding or eroding the ossicular chain. If left unaddressed, congenital cholesteatoma can result in erosion of the bony labyrinth, tegmen, or facial nerve resulting in significant patient morbidity. Surgical removal of congenital cholesteatoma is recommended, preferably when the lesions are small. When considering a surgical approach for the removal of congenital cholesteatoma, surgeons should consider the size and location of the lesion as well as the extent of expected reconstruction. Transcanal endoscopic ear surgery can successfully be used to manage congenital cholesteatoma limited to the tympanic cavity. Patient features that favor an endoscopic approach include a circumscribed, unruptured congenital cholesteatoma, intact external auditory canal wall, and absence of secondary complications such as labyrinthine fistula, middle fossa extension, or facial nerve paresis. We describe our endoscopic operative approach to remove congenital cholesteatoma and also highlight effective methods of tympanic reconstruction.

Surgery for Cholesteatomatous Labyrinthine Fistula

There is uncertainty regarding the ideal surgical management of cholesteatomatous labyrinthine fistulae. The objective was to review the published evidence to determine whether a difference exists in hearing outcome for cholesteatoma matrix removal or matrix exteriorization. Systematic MEDLINE and Web of Science searches identified publications describing hearing results after cholesteatoma matrix removal or matrix exteriorization. Three reviewers appraised the studies for quality, level of evidence, and extracted data. Fistula characteristics such as single-site, multisite, size, grade, and follow-up time were extracted for subanalyses, and when appropriate, data were pooled for statistical analysis. Twenty-eight articles met inclusion criteria, and the level of evidence was judged no better than level 3b. There was no difference in hearing preservation detected between matrix removal and exteriorization (87% for matrix removal, 95% CI, 0.82-0.90; 95% for exteriorization, 95% CI, 0.85-0.98). An analysis of the individual cohort studies that compared these groups directly did not show a difference in calculated odds ratio (OR), 0.96 (95% CI, 0.66-1.40). The level of evidence on which to base surgical decision making related to cholesteatomatous labyrinthine fistula is poor, and the data do not demonstrate significant differences in hearing outcomes based on surgical technique.

ダイビング後に生じためまい症例 : 内耳型減圧症と外リンパ瘻の鑑別について

ダイビング後に生じためまい症例 : 内耳型減圧症と外リンパ瘻の鑑別について

内耳瘻孔を伴う真珠腫性中耳炎の聴力成績

内耳瘻孔を伴う真珠腫性中耳炎の聴力成績

Cochlin-tomoprotein in patients with suspected perilymphatic fistula

Cochlin-tomoprotein in patients with suspected perilymphatic fistula

A case of perilymphatic fistula with pneumolabyrinthine caused by an old temporal bone fracture

A case of perilymphatic fistula with pneumolabyrinthine caused by an old temporal bone fracture

擤鼻による外リンパ瘻が原因で難聴を生じた２症例

擤鼻 (こうび, 鼻かみ) 後に生じた稀な外リンパ瘻の2症例を経験した. 外リンパ瘻とは, 急激な中耳圧の上昇によってアブミ骨が外れ, 外リンパが中耳に漏出する病態である. 変動する難聴が主訴の1症例はCoclin-tomoprotein (CTP) 検査が陽性で, 手術前に診断が確定し, 早期の鼓室形成術によって聴力は回復した. 激しいめまいと難聴の症例は, 内耳に空気塞栓を認め, 大量の外リンパ漏出による内耳障害は高度で, 鼓室形成術を施行するも診断の遅れのために聴力は回復しなかった. 術中のCTP検査は陰性で, すでに外リンパは枯渇するほど漏出したと推察した. CTP検査は外リンパ瘻の診断に有用だが, 検査のタイミングによって, 結果を解釈する必要があった. 外リンパ瘻は早期に診断して手術すれば回復する疾患なので, 迅速診断法の確立と, 疾患概念の医療関係者への周知が望まれる.

A rear case of labyrinthine fistula in a child

The authors described a clinical case of labyrinthine fistula in the region of the basal turn of the cochlea and labyrinth vestibule caused by noncholesteatomous chronic suppurative otitis media in a one year old child. The basal turn of the cochlea and labyrinth vestibule is a rare localization of labyrinthine fistula. A specific feature of this clinical observation is the fact that chronic suppurative otitis media was preceded by the measles otitis media, characterized with the development of destructive process in the middle ear.

The role of bone resorption in the etiopathogenesis of acquired middle ear cholesteatoma.

Cholesteatoma is a destructive squamous epithelial lesion of the temporal bone which gradually expands and leads to serious complications by destruction of nearby bony structures. Erosion caused by bone resorption of the ossicular chain and bony labyrinth may result in hearing loss, vestibular dysfunction, facial paralysis, labyrinthine fistulae and intracranial complications. The exact underlying cellular and molecular mechanism of bone resorption in acquired cholesteatoma still remains unexplained. Pubmed database and China National Knowledge Infrastructure were screened for articles focusing on bone resorption in acquired cholesteatoma. Osteoclast activation, pressure necrosis, acid lysis, enzyme mediation, inflammatory mediators and several newly discovered biomolecules are outlined as main theories behind bone resorption in acquired cholesteatoma, aiming to facilitate the development of potential therapeutic targets for preventing intracranial and extracranial complications caused by bone resorption in acquired middle ear cholesteatoma.

Autologous intratympanic blood patch for presumed perilymphatic fistulas.

To assess an alternative to bed rest and surgery for suspected perilymphatic fistulas using intratympanic blood injections. A review was conducted of patients' history, physical and audiometric data, before and after treatment by intratympanic blood injections performed from 2009 to 2015. Twelve ears were identified, with trauma associated with air travel, water sports or nose blowing. Ten of these cases had hearing loss, six had vestibular symptoms. Four cases had audiological and vestibular symptoms, two had vestibular symptoms only, and six had audiological symptoms only. Time-to-treat varied from 1 day to 30 days. Magnetic resonance imaging scans were obtained for five cases. Ten cases received steroids. Six out of seven cases showed improvement of hearing loss. Five cases showed positive fistula test results, four with documented resolution. Seven cases had full resolution of all symptoms, four had near-full resolution and one had no improvement. Intratympanic blood injections offer an effective alternative to conservative or surgical therapy. Advantages include sooner time-to-treat, lower financial costs and decreased psychosocial burdens. It allows a more flexible and liberal use of a potential definite treatment for perilymphatic fistula.

Risk of progressive hearing loss in untreated superior semicircular canal dehiscence.

Patients with incidental or minimally symptomatic superior semicircular canal dehiscence (SSCD) are usually observed, without surgical repair. However, it remains unknown whether a labyrinthine fistula of the superior semicircular canal is associated with progressive conductive or sensorineural hearing loss over time. Retrospective review at two tertiary care academic referral centers. Adults analyzed were diagnosed with SSCD by high-resolution temporal bone computed tomography and vestibular evoked myogenic potential testing and observed with a minimum of two sequential audiograms. Patients with other potential causes of hearing impairment were excluded. A total of 40 ears in 30 adult patients (median age: 59 years; 63% female) were analyzed. Median audiometric follow-up was 23 months (range 1-136 months). None experienced a sudden hearing loss over the follow-up period. In patients with audiometric follow-up of at least 20 months (median 34 months), the median change in air-conduction pure tone average and air-bone gap was 0.9 decibels (dB) per year (interquartile range [IQR] 0-2.1) and 0.7 dB per year (IQR 0-2.0), respectively. Speech discrimination scores did not differ when comparing median initial (100%) and median final (98%) scores (P = 0.77). There was no statistically significant change in bone-conduction thresholds at 0.5, 1, 2, and 4 kHz over the period of observation. The risk of progressive hearing loss with observed SSCD is low during short- and intermediate-term follow-up. Further studies are necessary to determine whether late hearing loss occurs. Such information may be critical toward patient counseling regarding the need for and timing of surgery. 4. Laryngoscope, 127:1181-1186, 2017.

Presence of osteoclasts in middle ear cholesteatoma: a study of undecalcified bone sections

Conclusions: Osteoclasts are unlikely to be involved in bone resorption in middle ear cholesteatoma.Objective: The authors searched for osteoclasts in undecalcified bone sections in patients with middle ear cholesteatoma to determine whether and to what extent these cells are involved in this disease.Methods: Twelve patients, eight men and four women, aged 30–87 years, who underwent tympanomastoidectomy were enrolled. Six patients had primary acquired middle ear cholesteatoma (cholesteatoma group) and the other six patients had other otologic diseases including otosclerosis, non-cholesteatomatous chronic otitis media, adhesive otitis media, perilymphatic fistula and ossicular malformation (control group). The scutum bone was collected during surgery, fixed with ethanol, stained with Villanueva bone stain, and embedded in methyl methacrylate. Five-micrometer-thick sections were prepared and examined under a polarizing microscope. Images were analyzed using a semiautomatic graphics system.Results: No osteoclasts were seen in any of the samples in either group. To avoid the risk of under-estimating the presence of osteoclasts, the number of osteoclasts was considered to be <1 in each sample, and the osteoclast density was calculated. The osteoclast densities in both the cholesteatoma and control groups were significantly lower than the sex- and age-matched standard value of the normal iliac cortical bone (p = .028).

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.

Pediatric sudden sensorineural hearing loss: Etiology, diagnosis and treatment in 20 children

Objectives1. To report our experience in children with sudden-onset sensorineural hearing loss (SSNHL). 2. To describe the etiology and management of children with SSNHL. MethodsRetrospective review of 20 children with SSNHL, from 2000 to 2013 at a tertiary pediatric facility. Patients had the following inclusion criteria: history of normal hearing, hearing loss occurring in less than 3 days, and audiogram documentation. ResultsThe average age of patients presenting with SSNHL is 11 years 3 months (22months-18years). Only 6 (30%) children presented prior to 2 weeks. Tinnitus (55%) was the most common associated symptoms followed by otalgia (25%), and vertigo (20%). Eight patients had bilateral hearing loss, 6 only right and 6 only left. Hearing loss severity ranged from profound (45%) being most common to mild. Etiology was unknown (30%), viral (25%), anatomic abnormality (25%), Meniere's disease (5%), autoimmune (5%), perilymphatic fistula (5%), and suppurative labyrinthitis (5%). Eight patients had initial treatment with oral steroids of which 50% had improvement on audiograms. Two patients underwent intratympanic injections, both showed improvement. Of the 12 patients with no treatment, only 1 had improved hearing. ConclusionsThe true incidence of pediatric SSNHL is not well established in our literature. Unique aspects of pediatric SSNHL are delayed presentation and higher percent of anatomic findings. In our study 70% presented more than 2 weeks after experiencing symptoms. Anatomic abnormalities are in 40% of patients. Hearing improvement occurred in 50% of children treated with oral steroids. Intratympanic steroid treatment is another option but may have practical limitation in the pediatric population.

Delayed Surgical Intervention in the Management of Perilymphatic Fistula

Delayed Surgical Intervention in the Management of Perilymphatic Fistula

Safe and unsafe CSOM with sensori neural hearing loss: Pathophysiological study

Chronic suppurative otitis media is the commonest cause of hearing loss. The deafness caused by chronic suppurative otitis media of safe type was usually considered to be purely of conductive type. With this background present study was planned to study safe and unsafe CSOM with sensori neural hearing loss with Pathophysiological study. One hundred patients of chronic suppurative otitis media with sensori neural hearing loss, either alone or more commonly, with mixed loss were studied in this series. The patients were carefully selected after proper history and careful examination to exclude the above mentioned criteria to rule out the other possible causes of sensori neural loss. Type of pathology in each of these ears was the main factor taken into consideration. Age group and duration of illness were also considered. In our study, 14% cases of unsafe chronic suppurative otitis media were found to be associated with labyrinthine fistula. This proved to be the commonest cause of sensori neural component in unsafe chronic suppurative otitis media followed by granulation over oval window (8%) and cholesteatoma extending to round window in 4 % of cases. In our study, 14% cases of unsafe chronic suppurative otitis media were found to be associated with labyrinthine fistula. This proved to be the commonest cause of sensori neural component in unsafe chronic suppurative otitis media.

Effect of Round Window Reinforcement on Hearing: A Temporal Bone Study With Clinical Implications for Surgical Reinforcement of the Round Window.

Round window reinforcement leads to conductive hearing loss. The round window is stiffened surgically as therapy for various conditions, including perilymphatic fistula and superior semicircular canal dehiscence. Round window reinforcement reduces symptoms in these patients. However, it also reduces fluid displacement in the cochlea and might therefore increase conductive hearing loss. Perichondrium was applied to the round window membrane in nine fresh-frozen, nonpathologic temporal bones. In four temporal bones cartilage was applied subsequently. Acoustic stimuli in the form of frequency sweeps from 250 to 8000 Hz were generated at 110 dB sound pressure level. A total of 16 frequencies in a 1/3-octave series were used. Stapes velocities in response to the acoustic stimuli were measured at equally spaced multiple points covering the stapes footplate using a scanning laser Doppler interferometry system. Measurements were made at baseline, after applying perichondrium, and after applying cartilage. At frequencies up to 1000 Hz perichondrium reinforcement decreased stapes velocities by 1.5 to 2.9 dB compared with no reinforcement (p value = 0.003). Reinforcement with cartilage led to a further deterioration of stapes velocities by 2.6 to 4.2 dB at frequencies up to 1000 Hz (p value = 0.050). The higher frequencies were not affected by perichondrium reinforcement (p value = 0.774) or cartilage reinforcement (p value = 0.644). Our results seem to suggest a modest, clinically negligible effect of reinforcement with perichondrium. Placing cartilage on the round window resulted in a graded effect on stapes velocities in keeping with the increased stiffness of cartilage compared with perichondrium. Even so, the effect was relatively small.

Hydrops of the organ of Corti

Objectives: The authors would like to confirm a fluid pathway from the scala tympani to the organ of Corti, and to observe its morphological changes.Methods: A staining solution for succinic dehydrogenase was perfused with phenazine methosulfate in the scala tympani of living guinea pigs (n = 5) under deep anesthesia. After fixation, the cochleas were eventually embedded in epon. Sections were observed under a light microscope.Results: Blue-stained tissue is indicative of the pathway taken by the solution. The staining solution entered the organ of Corti through Hensen-Deiters’ slit. The slit widened and Hensen’s cells were pushed laterally. A new space was formed medial to Hensen’s cells. Cortilymphatic hydrops developed.Conclusion: The Hensen-Deiters’ slit is a pathway of a certain staining solution from the scala tympani to inside the organ of Corti of the guinea pig. The influx of the fluid pushes Hensen’s cells laterally and upward, resulting in a formation of hydrops of the organ of Corti or cortilymphatic hydrops. The hydrops is observed in animals with experimental perilymphatic fistula and with viral labyrinthitis. At the end stage of the hydrops, only the surface of the organ of Corti remains as a thin layer without any cellular elements.

Correlation between hearing results, CT-scan images and intraoperative findings in cholesteatoma related labyrinthine fistula

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