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Perilymph fistula: an important cause of deafness and dizziness in children.

This study underscores the importance of considering a perilymph fistula, an abnormal communication between the inner and middle ear, in any child with a progressive sensorineural hearing loss. A concomitant complaint of intermittent dizziness or observed spells of imbalance (56%) is another indication of the disease. Six of 16 patients (37.5%) with proven fistulas were less than 2 years of age at the onset of symptoms. Unless the fistula is identified and repaired by grafting, the ear may become totally deaf. This would be disastrous in the presence of bilateral fistulas, which occur commonly in children (56% of patients had symptoms of bilateral disease). Our experience in recognizing and treating fistulas and thereby saving and restoring hearing is described.

Adolescent↗

[Perilymph fistulas--symptoms and indications for tympanoscopy].

In the last two years thirteen cases of perilymph fistula were treated by tympanoscopy and covering with connective tissue. Recovery of hearing was not observed in 4 totally deaf ears, but improvement was recorded in each of 9 cases with impaired hearing. Vertigo disappeared in 10 of 11 cases without any correlation with the degree of hearing loss. We perform tympanoscopy in suspected perilymph fistula, because prognosis is uncertain, spontaneous recovery with remaining defects may induce ménièriform syndrome, and pathway of infection should be closed. The diagnosis of a perilymph fistula must be solidified in cases of sudden deafness with vertigo, sudden hearing loss changing to the worse during medical treatment, sudden hearing loss without recovery during medical treatment, sudden hearing loss and subsequent onset of vertigo.

Adult↗

Comparison of endoscopic and surgical explorations for perilymphatic fistulas.

A history suggestive of perilymphatic fistula (PLF) often prompts repeated tympanostomies to establish a diagnosis and perform a repair. Two patients having multiple previous explorations for perilymphatic fistulas were reoperated, comparing endoscopic and open surgical methods. A third patient with a history consistent with PLF also underwent dual assessment. Endoscopic exploration of the middle ear was performed through a myringotomy and, immediately after, by elevation of a tympanotomy flap. The endoscopic examinations were thorough yet revealed no evidence of perilymphatic fistula; however, the surgical approaches revealed pooling in the oval windows consistent with perilymphatic fistula. These findings were video documented. Recurrent and primary fistulas may be the result of artifact, such as injected anesthetic agents and transudates introduced during surgical explorations, which may interfere with an accurate diagnosis of perilymphatic fistula. Endoscopy of the middle ear is recommended as one method to minimize errors in diagnosis.

Adult↗

Validity of spontaneous perilymphatic fistula.

In response to recent articles questioning the validity of some perilymphatic fistula diagnoses, another small series is presented, reconfirming the enigmatic clinical appearance of perilymphatic fistula. Histologic evidence and experimental pathophysiology that explain the varied symptoms are reviewed. A pressed-fat fibrin-glue technique that seems to prevent recurrences is described, as well as a technique that may be helpful in confirming the presence of inapparent fistulas in surgery. Argument is advanced that this is a safe, minor surgical procedure helpful in alleviating vestibular and/or auditory symptoms in more than 70% of patients.

Cochlear Diseases↗

[Bifenestration--a hearing-improving operation in severe ear malformations].

Bifenestration as an operation for hearing improvement is described in a case of congenital absence of the oval and round window. Besides the classical fenestration of the horizontal semicircular duct, an artificial round window was performed in the middle ear. Due to the acoustic resonance of the mastoid cavity, the resulting conductive loss in the higher frequencies was only 20-30 dB.

Adult↗

Inner ear decompression sickness combined with a fistula of the round window. Case report.

Inner ear barotrauma with rupture of the round or oval window secondary to diving and decompression sickness (DCS) of the inner ear can be a difficult diagnosis to differentiate. The dive profile or associated elements of DCS will often confirm the diagnosis. Occasionally, diagnosis is made during recompression or during operation. The differential diagnosis is important, since immediate recompression is indicated for inner ear DCS, while it is contraindicated in cases of inner ear barotrauma. We have found no cases reported in the world literature in which both diseases have been diagnosed and proven simultaneously. We present a case of a diver who developed DCS with inner ear manifestations complicated by a round window fistula. Treatment and clinical outcome are discussed along with a brief review of the suspected cause.

Audiometry↗

[A case report of bilateral perilymph fistula in an adult, with literature review].

Adult bilateral perilymph fistula (PLF) is rare. We report a case of a 55-year-old man who suffered from sudden bilateral hearing loss after blowing his nose. On the first day, he also had bilateral tinnitus but no vertigo. However, he gradually developed vertigo, and nystagmus to the left side began to appear in the following days. Exploratory bilateral tympanotomies were performed and perilymph fistulas were observed at the round window niche in both ears. The round windows were obliterated with fascia of the temporal muscles. After the operation no changes were found in both sides on a pure tone audiogram even at a one-year follow-up. The literature was reviewed and several features were statistically analyzed. In the adult bilateral PLF group, the proportion of the cases in which the cause of PLF was clear was significantly higher than that in the adult unilateral PLF group, but the other clinical features were almost the same. The results suggest that in adults the occurrence of bilateral and unilateral PLF could depend on the level of pressure applied to the middle or inner ear. On the other hand, in the child PLF group, the ratio of bilateral involvement was considerably higher than that in the adult PLF group, and in most cases the PLF was associated with middle and/or inner ear malformations. Healing in the child bilateral PLF group was poor than that in the adult PLF group. Those results suggest that the etiology of bilateral PLF in children may be considerably different from that of adult PLF.

Age Factors↗

Ultrastructural organization of the round window membrane in the infant human middle ear.

Ultrastructural studies of the round window membrane in infants revealed that the membrane is made up of three layers. The relatively wide outer epithelial layer consists of 3-4 cell layers and is formed by cuboidal and round cells. The outer two or three cell layers contain mainly rough endoplasmic reticulum and secretory granules, indicative of a secretory function of the epithelial cell layer of middle ear mucosa. Tonofilaments occur mainly in the more electron-dense epithelial cell layers which makes their mechanical role more probable. The connective tissue layer is relatively narrow and contains fibrocytes, much collagen and relatively few elastic fibres. The inner epithelial layer is built up of elongated cells containing rough endoplasmic reticulum cisternae in their cytoplasm.

Cochlea↗

Oxygenation through the round window membrane and the inner ear function.

The effect of oxygenation through the round window membrane was estimated using polarographic technique and the combined action potential of the inner ear. The calculated amount of oxygen permeable through the round window membrane (14% in room air and 80% in pure oxygen) revealed to have very limited effect in maintaining the inner ear potential. Carbogen inflation in maintaining the N1 potential during asphyxia was significantly effective only at 8 kHz. Oxygen supply by this route, however, may enable the inner ear tissue alive even in misery perfusion and recover the high tone potential as a therapy of otitis media with effusion.

Animals↗

Treatment and management of perilymphatic fistula: a New Hampshire experience.

From August of 1975 to June of 1990 the author managed 45 cases of spontaneously occurring perilymphatic fistula. Twenty-nine of the 45 cases were reported in 1988. In the series there were eight patients with bilateral involvement, seven with involvement of both oval and round window in the same ear, and seven with fistulas believed to be of congenital origin. Seventeen patients (38%) required revision surgery. Occasionally adjunct modalities (endolymphatic shunt, labyrinthectomy, blocking a cochlear aqueduct, and streptomycin ablation) were used for symptom control. Diagnosis, treatment, and management of these patients is discussed.

Adolescent↗

Tolerance of membranous inner ear structures to pressure.

Pressure tolerance of round and oval labyrinthine windows and a cochlear segment represented by a part of the cochlear duct were investigated in experiments on cadaveric human temporal bones. The cochlear segment is less resistant than both the windows. Inner ear spaces are protected from pressure changes in the surroundings of the temporal bone by a system of narrow and comparatively long connections and some of them include also other structures strengthening the protective function (the valve described near the place where the cochlear aqueduct leads to scala tympani). Experiments with guinea pigs showed significance of inner ear integrity in protection from pressure changes. Tolerance of both windows was significantly higher in live animals than in cadaveric guinea pig bullae. Pressure tolerance of the windows was in turn higher in bullae than in isolated inner ear labyrinth. Conclusions were arrived at on the basis of the above facts that the inner ear is considerably resistant to pressure changes around it under normal circumstances. Ruptures in its parts appear only under special conditions and a number of factors participate in them. Window ruptures accompany most frequently injuries in other inner ear structures to which different degrees of hearing loss correspond. An isolated injury of windows is, from this viewpoint, rare even though it can be found and treated surgically best.

Animals↗

Round window reflex and oval window fistulae.

The round window reflex is normally absent in stapedial fixation. In this study, six cases of oval window fistula are described in which the round window reflex was absent. This is probably because there is insufficient pressure transmitted to the round window membrane through the perilymph because of leakage from the oval window. This is considered as an important diagnostic sign of oval window/stapes footplate ligament disruption.

Cochlea↗

T cell subsets in round window membrane after middle ear immunostimulation.

In secondary middle ear immune response, kinetics of immunocytes, especially T cell subsets, was examined in the round window membrane (RWM) using immunohistochemical methods. Healthy BALB/c mice and keyhole limpet hemocyanin (KLH) antigen were employed in this study. The inflammatory responses of the RWM and middle ear were investigated after antigen challenge into the middle ear bulla. We used antibodies against murine macrophages and granulocytes (anti-Mac-1), murine helper/inducer T cells (anti-Lyt-1 and -L3T4), murine suppressor/cytotoxic T cells (anti-Lyt-2), murine interleukin-2 receptor (7D4), murine immunoglobulins (anti-IgG, -IgM and -IgA) and KLH. In the RWM and middle ear mucosa, inflammatory cells were observed at 6 hours, peaking on days 3-7, whereas these cells were rarely seen in the scala tympani of the basal turn. Luminal effusion with an enormous infiltration of inflammatory cells, which consisted mainly of Mac-1 cells, IgG cells and IgM cells, was observed in the middle ear cavity on days 1-7 post antigen challenge. In the inflamed RWM, Mac-1 cells were the predominant cell type followed by helper T cells, interleukin 2 receptor positive cells and IgG positive cells, though IgM, IgA and Lyt-2 positive cells were rarely observed after antigen challenge. Our results suggest that RWM has the ability to protect inner ear by cellular immune response through activated helper T cells and Mac-1 cells.

Animals↗

[The "window-fistula symptom" in lesions of the round and oval window].

22 tympanoscopies in suspected perilymph fistulas with clinical symptoms such as sudden hearing loss, tinnitus, vertigo, fluctuating hearing etc., histories and intraoperative findings were registered. In some cases a remarkable interval between the implicated event and the clinical manifestation of the symptoms was noticed. Besides on case history and audiometric data the diagnosis was based on vestibular investigations. In 14 of 22 cases the window-fistula-symptom was positive. The window-fistula-symptom is defined as a provocation nystagmus with maximal intensity in the lateral head position lying on the affected ear and in the head hanging position with the head rotated to the affected side. After abandoning these positions the nystagmus is decreased. Mostly the nystagmus is directed to the healthy side. With regard to the preoperative and postoperative findings a generous indication for tympanoscopy in suspected fistulas of the round and oval window membrane is recommended.

Cochlea↗