Signal and morphological changes in the endolymph of patients with vestibular schwannoma on non-contrast 3D FLAIR at 3 Tesla.

BACKGROUND: Non-contrast FLAIR revealed increased signal within the inner ear in patients with vestibular schwannoma, which is generally assumed to occur in the perilymph; however, the majority of previous studies did not differentiate between the endolymph and perilymph. Therefore, endolymph signal changes have not yet been investigated in detail. The purpose of the present study was three-fold: (1) to assess perilymph signal changes in patients with vestibular schwannoma on heavily T2-weighted (T2W) 3D FLAIR, also termed positive perilymphatic images (PPI), (2) to evaluate signal and morphological changes in the endolymph on PPI, and (3) to establish whether vertigo correlates with the signal intensity ratios (SIR) of the vestibular perilymph or vestibular endolymphatic hydrops. METHODS: Forty-two patients with unilateral vestibular schwannoma were retrospectively recruited. We semi-quantitatively and qualitatively evaluated the perilymph signal intensity on the affected and unaffected sides. We also quantitatively examined the signal intensity of the vestibular perilymph and assessed the relationship between vertigo and the SIR of the vestibular perilymph on the affected side. We semi-quantitatively or qualitatively evaluated the endolymph, and investigated whether vestibular hydrops correlated with vertigo. RESULTS: The perilymph on the affected side showed abnormal signal more frequently (signal intensity grade: overall mean 1.45 vs. 0.02; comparison of signal intensity: overall mean 36 vs. 0 cases) and in more parts (the entire inner ear vs. the basal turn of the cochlea and vestibule) than that on the unaffected side. No significant difference was observed in the SIR of the vestibular perilymph with and without vertigo (5.54 vs. 5.51, p = 0.18). The endolymph of the vestibule and semicircular canals showed the following characteristic features: no visualization (n = 4), signal change (n = 1), or vestibular hydrops (n = 10). A correlation was not observed between vestibular hydrops and vertigo (p = 1.000). CONCLUSIONS: PPI may provide useful information on signal and morphological changes in the endolymph of patients with vestibular schwannoma. Further research is warranted to clarify the relationship between vertigo and the MR features of the inner ear.

The role of monocytes and macrophages in the dynamic permeability of the blood-perilymph barrier.

The blood-perilymph barrier serves a critical role by separating the components of blood from inner ear fluids, limiting traffic of cells, proteins and other solutes into the labyrinth, and allowing gas (O2-CO2) exchange. Inflammation produces changes in the blood-perilymph barrier resulting in increased vascular permeability. It is commonly thought that compromise of the blood-inner ear barrier would lead to hearing impairment through loss of the endocochlear potential (EP). In fact, the effect of increasing cochlear vascular permeability on hearing function and EP is poorly understood. We used a novel method to measure the integrity of the blood-perilymph barrier and demonstrated the effects of barrier compromise on ABR threshold and EP. We also investigated the contribution of CX3CR1 cochlear macrophages and CCR2 inflammatory monocytes to barrier function after systemic exposure to lipopolysaccharide (LPS). We found that systemic LPS induced a profound change in vascular permeability, which correlated with minimal change in ABR threshold and EP. Macrophage depletion using CX3CR1-DTR mice did not alter the baseline permeability of cochlear vessels and resulted in preservation of barrier function in LPS-treated animals. We conclude that cochlear macrophages are not required to maintain the barrier in normal mice and activated macrophages are a critical factor in breakdown of the barrier after LPS. CCR2 null mice demonstrated that LPS induction of barrier leakiness occurs in the absence of CCR2 expression. Thus, enhanced aminoglycoside ototoxicity after LPS can be linked to the expression of CCR2 in inflammatory monocytes, and not to preservation of the blood-perilymph barrier in CCR2 knockout mice.

Oval Window Temperature Changes in an Endoscopic Stapedectomy.

OBJECTIVES: Following the initial use of endoscopes in otology, the pros and cons of these instruments have been questioned increasingly. These instruments cause an increase in temperature that needs to be investigated. In this study, the authors aimed to investigate the temperature increase caused by endoscopes and light sources in the perilymph by performing a stapedotomy in an animal model under anesthesia. STUDY DESIGN: The study was performed in a guinea pig model. METHODS: In the animal model, a simulated otologic stapes surgery was performed at room temperature. The body temperatures of the guinea pigs were monitored; the temperature increase caused by the 0-degree rigid endoscopes with diameters of 3 and 4  mm as well as the light sources, including halogen, light-emitting diode (LED), and xenon lamps, were monitored following the stapedotomy to measure and document the continuous temperature increase in the perilymph using sensors placed at the oval window. RESULTS: Rigid endoscopes cause a temperature increase in the tympanum regardless of their diameter when used with xenon and halogen light sources. The LED light caused a relatively small temperature increase. CONCLUSIONS: The endoscopic instruments used in the stapes operation caused a temperature increase in the oval window. The authors concluded that this heat could easily be transmitted to the cochlea by the perilymph, which has obstructed contact with the outer environment following stapedomy, resulting in neurosensorial damage.

The enigmatic root cell - emerging roles contributing to fluid homeostasis within the cochlear outer sulcus.

Despite their curious morphology prompting numerous hypotheses of their normal function, the root cells lining the cochlear outer sulcus have long evaded physiological characterization. A growing body of evidence now suggests that they regulate the solute content of the endolymph and/or the perilymph, and may be essential in safe-guarding the global homeostasis of the cochlea. Immuno-labeling experiments have demonstrated polarized expression of key ion transport proteins, and recent electrophysiological recordings have identified specific membrane conductances. These studies have painted a clearer picture of how this unusual cell type may contribute to the maintenance of sound transduction, and how they may be central to pathological processes associated with various forms of hearing loss. This article is part of a Special Issue entitled "Annual Reviews 2013".

Influence of an intraoperative perilymph gusher on cochlear implant performance in children with labyrinthine malformations.

OBJECTIVES: To assess the effect of an intraoperative perilymph fluid gusher during cochlear implantation on speech perception abilities in pediatric patients with labyrinthine anomalies. SETTING: Tertiary care academic referral center. METHODS: Seventy subjects with labyrinthine malformations who received a cochlear implant were identified in our pediatric cochlear implant database. In 30 cases, an intraoperative perilymph fluid gusher was encountered during surgery. Fifteen children with GJB2 positive hereditary hearing loss served as controls. Multiple speech perception measures were obtained with the cochlear implant. The best score for each subject over time was determined as a speech reception index in quiet. This index was compared among groups and malformation types. RESULTS: The speech reception index in quiet demonstrated overall good performance scores of cochlear implantation in children with incomplete partitioning/enlarged vestibular aqueduct type malformations. Children with hypoplastic malformations, on the other hand, showed variable outcomes with many children demonstrating only limited long-term speech discrimination abilities. The presence or absence of a perilymph gusher did not significantly influence results after cochlear implantation. CONCLUSION: This report documents the variable outcomes of pediatric cochlear implantation in children with inner ear malformations. More importantly, anatomic parameters, such as the classification of the anomaly and the presence of a viable cochlear nerve, seem to influence performance measures substantially. The presence of a perilymph gusher did not influence outcomes in both hypoplastic and incomplete partitioning/enlarged vestibular aqueduct type malformations. LEVEL OF EVIDENCE: 2b Individual retrospective cohort study.

Differences in the perilymph fluid stimulation before and after experimental stapedotomy.

A stapedotomy surgery using a piston stapes prosthesis significantly modifies the perilymph fluid stimulation level and always leads to alteration of conditions in sound transmission through the cochlea. This paper shows the results of non-contact measurements of the stapes head velocity, a Teflon piston stapes prosthesis velocity and round window velocity conducted in freshly harvested human cadaver temporal bone specimens. The vibration patterns were measured within the frequency range of 0.4-10 kHz at the sound pressure level of 90 dB administered to the external auditory canal in the same specimen before and after experimental stapedotomy. It was shown that the vibrations of the stapes Teflon piston prosthesis and the physiological stapes are similar and approximately five-fold lower amplitude of the round window membrane vibrations compared to a physiologic situation is caused by piston shape of the stapes prosthesis. The results in this report are the part of a larger study designed to develop a new type of chamber stapes or whole middle ear prosthesis.

[Traumatic perilymphatic fistulae : about 13 cases]. / Fistules péri lymphatiques post traumatiques : a propos 13 cas.

BACKGROUND: Post-traumatic peri-lymphatic fistulas have been described following ear and temporal bone injury, particularly in the setting of temporal bone fractures. The symptoms and signs of perilymphatic fistulae (PLF) are very varied and frequently misleading. The diagnosis can be suspected on the bases of the clinical and the audiometrial findings. Indications for exploratory surgery in cases of trauma are vague and not well described. AIM: To assess the principal clinical and radiologic signs of PLF. METHOD: Study of 13 patients with different symptoms of posttraumatic peri-lymphatic fistulae. RESULTS: Ten patients had vertigo, and 2 presented otoliquorreha. Two patients had tympanic perforation. Nine patients presented neurosensorinal hearing loss and 5 were completely deaf. A CT Scann was realized in 12 cases and showed the fracture in 10 cases (91%) with a pneumolabyrinth in 4 cases. Medical and postural treatment was indicated for all the patients then a surgery was indicated in all of them in an average wait of 4 months realizing an ear exclusion in one case and a filling-up for 12 patients. Vertigo improved in 10 cases and the hearing loss in 2 cases. CONCLUSION: The diagnostic of perilymphatic fistulae is not easy. The trauma and the clinical signs can help but the confirmation is surgical. The indication of surgery and its timing are still discussed.

Revision stapedotomy: operative findings and hearing results. A prospective study of 652 cases from the Otology-Neurotology Database.

OBJECTIVE: To identify the causes of failure of primary stapes surgery and to evaluate the hearing results of revision stapes surgery in a consecutive series of 652 cases. STUDY DESIGN: Prospective nonrandomized clinical study. SETTING: Tertiary referral center. PATIENTS: Six hundred thirty-four patients who underwent 652 consecutive revision stapes operations from April 1992 to December 2007 were enrolled in this study. MAIN OUTCOME MEASURES: Preoperative and postoperative audiometric evaluation using conventional audiometry, namely, air-bone gap (ABG), bone-conduction thresholds, and air-conduction thresholds, were assessed. Postoperative audiometry was performed at 3, 6, 9, 12, 18, and 24 months and then annually for 15 years. RESULTS: The most frequently identified reason for primary surgery failure was incus erosion (27.6%) and prosthesis displacement (18.2%). The postoperative ABG was closed to 10 dB or less and 20 dB or less in 63.4 and 74.6% of cases, respectively. The mean 4-frequency postoperative ABG was 11.5 dB as compared with 28 dB preoperatively (mean difference, 16.5 dB; 95% confidence interval [CI], 15.1-17.9 dB, p < 0.0001). The mean 4-frequency postoperative air-conduction thresholds were 45.7 dB compared with 58.7 dB preoperatively (mean difference, 13 dB; 95% CI, 11.4-14.6 dB, p < 0.0001). The mean 4-frequency postoperative bone-conduction thresholds were 34 dB compared with 30.6 dB preoperatively (mean difference, -3.5 dB; 95% CI, -4.4 to -2.5 dB, p < 0.0001). A significant postoperative sensorineural hearing loss (>15 dB) was observed in 2.9% of cases in this series. CONCLUSION: Improvement of a conductive hearing loss after initial unsuccessful primary or revision stapes surgery can be accomplished with further revision but is occasionally modest.

The performance of Cochlin-tomoprotein detection test in the diagnosis of perilymphatic fistula.

BACKGROUND: Perilymphatic fistula (PLF), defined as an abnormal communication between the inner and middle ear, presents with a symptomatology of hearing loss and vestibular disorder that is indistinguishable from a number of other inner ear diseases. Methods of diagnosis remain controversial. We have previously shown that Cochlin-tomoprotein (CTP) is selectively detected in the perilymph. To establish a definite diagnostic test for PLF using CTP as a biochemical marker, we examined the diagnostic performance of the CTP detection test. METHODS: CTP detection test was performed by Western blot using recombinant human CTP (rhCTP) as a spiked standard. We evaluated the specificity of the CTP detection test by testing non-PLF cases. To describe the limitations of the test, we tested samples from patients with middle ear infection. We also studied the stability of CTP protein by storing the samples at room temperature (25 degrees C) or 4 degrees C for 55 days. The effects of repeated freezing and thawing were also evaluated. Serially diluted perilymph was tested to find out the detection limit of CTP. FINDINGS: We have established a standardized CTP detection test using high (0.27 ng) and low (0.13 ng) spiked standards of rhCTP in Western blotting. Middle ear lavages (MEL) from 54 of 55 non-PLF cases were negative in the CTP detection test, i.e. the specificity of the test is 98.2%. MEL from 43 out of 46 cases with chronic suppurative otitis media or middle ear cholesteatoma were negative for CTP. CTP is a stable protein and detection was not affected by the storage, or freezing and thawing. The detection limit of perilymph was 0.161 microl/lane in an average of 5 samples. INTERPRETATION: CTP is a stable perilymph-specific protein, and this CTP detection could be the first clinically established diagnostic tool to detect PLF with a high specificity. PLF is surgically correctable by sealing the fistula. Appropriate recognition and treatment of PLF can improve hearing and balance in afflicted patients.

Fluid dynamics vascular theory of brain and inner-ear function in traumatic brain injury: a translational hypothesis for diagnosis and treatment.

It is hypothesized that in all traumatic brain injury (TBI) patients with a clinical history of closed or penetrating head injury, the initial head trauma is associated with a vibratory sensation and noise exposure, with resultant alteration in vascular supply to the structures and contents of the fluid compartments of brain and ear (i.e., the fluid dynamics vascular theory of brain-inner-ear function [FDVTBE]). The primary etiology-head trauma-results in an initial fluctuation, interference, or interaction in the normal fluid dynamics between brain and labyrinth of the inner ear, with a resultant clinical diversity of complaints varying in time of onset and severity. Normal function of the brain and ear is a reflection of a normal state of homeostasis between the fluid compartments in the brain of cerebrospinal fluid and perilymph-endolymph in the labyrinth of the ear. The normal homeostasis in the structures and contents between the two fluid compartment systems--intracerebral and intralabyrinthine--is controlled by mechanisms involved in the maintenance of normal pressures, water and electrolyte content, and neurotransmitter activities. The initial pathophysiology (a reflection of an alteration in the vascular supply to the brain-ear) is hypothesized to be an initial acute inflammatory response, persistence of which results in ischemia and an irreversible alteration in the involved neural substrates of brain-ear. Clinically, a chronic multisymptom complex becomes manifest. The multisymptom complex, individual for each TBI patient regardless of the diagnostic TBI category (i.e., mild, moderate, or severe), initially reflects processes of inflammation and ischemia which, in brain, result in brain volume loss identified as neurodegeneration and hydrocephalus ex vacuo or an alteration in cerebrospinal fluid production (i.e., pseudotumor cerebri) and, in ear, secondary endolymphatic hydrops with associated cochleovestibular complaints of hearing loss, tinnitus, vertigo, ear blockage, and hyperacusis. The FDVTBE integrates and translates a neurovascular hypothesis for Alzheimer's disease to TBI. This study presents an FDVTBE hypothesis of TBI to explain the clinical association of head trauma (TBI) and central nervous system neurodegeneration with multisensory complaints, highlighted by and focusing on cochleovestibular complaints. A clinical case report, previously published for demonstration of the cerebrovascular medical significance of a particular type of tinnitus, and evidence-based basic science and clinical medicine are cited to provide objective evidence in support and demonstration of the FDVTBE.

A novel implantable hearing system with direct acoustic cochlear stimulation.

A new implantable hearing system, the direct acoustic cochlear stimulator (DACS) is presented. This system is based on the principle of a power-driven stapes prosthesis and intended for the treatment of severe mixed hearing loss due to advanced otosclerosis. It consists of an implantable electromagnetic transducer, which transfers acoustic energy directly to the inner ear, and an audio processor worn externally behind the implanted ear. The device is implanted using a specially developed retromeatal microsurgical approach. After removal of the stapes, a conventional stapes prosthesis is attached to the transducer and placed in the oval window to allow direct acoustical coupling to the perilymph of the inner ear. In order to restore the natural sound transmission of the ossicular chain, a second stapes prosthesis is placed in parallel to the first one into the oval window and attached to the patient's own incus, as in a conventional stapedectomy. Four patients were implanted with an investigational DACS device. The hearing threshold of the implanted ears before implantation ranged from 78 to 101 dB (air conduction, pure tone average, 0.5-4 kHz) with air-bone gaps of 33-44 dB in the same frequency range. Postoperatively, substantial improvements in sound field thresholds, speech intelligibility as well as in the subjective assessment of everyday situations were found in all patients. Two years after the implantations, monosyllabic word recognition scores in quiet at 75 dB improved by 45-100 percent points when using the DACS. Furthermore, hearing thresholds were already improved by the second stapes prosthesis alone by 14-28 dB (pure tone average 0.5-4 kHz, DACS switched off). No device-related serious medical complications occurred and all patients have continued to use their device on a daily basis for over 2 years.

Effects of SERCA and PMCA inhibitors on the survival of rat cochlear hair cells during ischemia in vitro.

An important mechanism underlying cochlear hair cell (HC) susceptibility to hypoxia/ischemia is the influx of Ca(2+). Two main ATP-dependent mechanisms contribute to maintaining low Ca(2+) levels: uptake of Ca(2+) into intracellular stores via smooth endoplasmic reticulum calcium ATPase (SERCA) and extrusion of Ca(2+) via plasma membrane calcium ATPase (PMCA). The effects of the SERCA inhibitors thapsigargin (10 nM-10 microM) and cyclopiazonic acid (CPA; 10-50 microM) and of the PMCA blockers eosin (1.5-10 microM) and o-vanadate (1-5 mM) on inner and outer hair cells (IHCs/OHCs) were examined in normoxia and ischemia using an in vitro model of the newborn rat cochlea. Exposure of the cultures to ischemia resulted in a significant loss of HCs. Thapsigargin and CPA had no effect. Eosin decreased the numbers of IHCs and OHCs by up to 25 % in normoxia and significantly aggravated the ischemia-induced damage to IHCs at 5 and 10 microM and to OHCs at 10 microM. o-Vanadate had no effect on IHC and OHC counts in normoxia, but aggravated the ischemia-induced HC loss in a dose-dependent manner. The effects of eosin and o-vanadate indicate that PMCA has an important role to play in protecting the HCs from ischemic cell death.

Coenzyme Q10 does not protect cochlear hair cells from death in the ischemic organotypic culture.

OBJECTIVE: To evaluate in vitro the effect of coenzyme Q10 (CoQ(10)) on ischemia-induced hair cell death. STUDY DESIGN: Organotypic cochlear cultures of newborn rats were subjected to ischemia with and without CoQ(10). RESULTS: Addition of CoQ(10) has not prevented HC loss. CONCLUSION: CoQ(10) seems to protect against only certain modes of cell death.

The influence of the footplate-perilymph interface on postoperative bone conduction.

In a prospective study, 165 total stapedectomies and 152 small fenestra stapedotomies were performed by three experienced surgeons between 2001 and 2003. In total stapedectomy, a self-made Schuknecht steel wire connective tissue prosthesis, and in stapedotomy, a 0.6-mm platinum wire Teflon piston was used. The pre- and postoperative bone conduction thresholds were compared at the frequencies 250 Hz, 500 Hz, 1 kHz, 1.5 kHz, 2 kHz, 3 kHz and 4 kHz. The postoperative bone conduction between 250 Hz and 3 kHz was significantly better in the total stapedectomy group than in the stapedotomy group. At 4 kHz, both groups showed a slight decrease in bone conduction but the difference was not statistically significant. Therefore, especially in cases with preoperative moderate sensorineural hearing loss, we recommend total stapedectomy using a Schuknecht steel wire connective tissue prosthesis, which offers a stapes-perilymph interface similar to the normal stapes.

Local overpressure treatment reduces vestibular symptoms in patients with Meniere's disease: a clinical, randomized, multicenter, double-blind, placebo-controlled study.

OBJECTIVES: To evaluate the efficacy of a new device, the Meniett, in the treatment of Meniere's disease. The device delivers pressure pulses to the middle ear through a ventilating tube in the tympanic membrane at a frequency of 6 Hz for 0.6 second. After rising to a pressure level of 1.2 kPa, the pressure oscillates between 0.4 and 1.2 kPa. It is believed that the pressure changes are conveyed to the inner ear, inducing a transport of fluids via the pressure outlets and thus reducing the endolymphatic hydrops. STUDY DESIGN: A clinical, randomized, multicenter, double-blind, placebo-controlled study. A total of 40 patients were included that had active Meniere's disease according to American Academy of Otolaryngology-Head and Neck Surgery criteria, aged between 20 and 65 years, with a history of at least eight attacks during the past year. After insertion of the ventilation tube, the patients should have had attacks of vertigo for 2 months before entering the study. OUTCOME MEASURES: Primary study endpoints were change in frequency of vertigo, change of functionality profile, and change in patient perception of vertigo (visual analogue scale); secondary endpoints were perception of tinnitus, aural pressure, and hearing, as well as an audiologic evaluation of hearing before and after the treatment period. RESULTS: The functionality level improved statistically significantly in the active group compared with the placebo group (p=0.0014), as did the visual analogue scale evaluation of vertigo (p=0.005). There was a trend toward a reduction of the frequency of vertiginous attacks that was not significant (p=0.090). With regard to the secondary endpoints, there was no statistical difference between active and placebo groups. CONCLUSION: Local overpressure treatment is a novel treatment that is noninvasive, nondestructive, and safe. It significantly reduces vestibular symptoms in patients with Meniere's disease. The Meniett was cleared by the Food and Drug Administration in 2000.

Cochlear activation at low sound intensities by a fluid pathway.

In order to assess the mechanisms responsible for cochlear activation at low sound intensities, a semi-circular canal was fenestrated in fat sand rats, and in other experiments a hole was made in the bone over the scala vestibuli of the first turn of the guinea-pig cochlea. Such holes, which expose the cochlear fluids to air, provide a sound pathway out of the cochlea which is of lower impedance than that through the round window. This should attenuate the pressure difference across the cochlear partition and thereby reduce the driving force for the base-to-apex traveling wave along the basilar membrane. The thresholds of the auditory nerve brainstem evoked responses (ABR) and of the cochlear microphonic potentials were not affected in the fenestration experiments. In addition, holes in the scala vestibuli of the first turn did not cause ABR threshold elevations. These results contribute further evidence that at low sound intensities the outer hair cells are probably not activated by a base-to-apex traveling wave along the basilar membrane. Instead it is possible that they are excited directly by the alternating condensation/rarefaction fluid pressures induced by the vibrations of the stapes footplate. The activated outer hair cells would then cause the localized basilar membrane movement.

Hyperbaric compression in the guinea pig with perilymph fistula.

OBJECTIVE: The study goal was to evaluate the effects of hyperbaric therapy on the auditory and vestibular function of guinea pigs compromised by perilymph fistula (PLF). METHODS: Twenty-four pigmented guinea pigs underwent induction of bilateral eustachian tube dysfunction before the creation of a unilateral PLF. Half of the animals were randomly assigned to receive immediate hyperbaric compression treatment of 4 "dives" each. Hearing was tested electrocochleographically, and signs of vestibulopathy were recorded before and after compression. RESULTS: After accounting for the effects of PLF, compression was not associated with significant hearing loss (P = 0.5411). Vestibulopathy was seen only in ears with PLF, and its incidence was similar for compression and noncompression groups. CONCLUSION: Hyperbaric compression does not compromise auditory or vestibular function in guinea pig ears with experimental PLF. SIGNIFICANCE: Our observations support the relative safety of recompression in patients with PLF.

[Perilymphatic effusion as a complication of otosclerosis]. / Geyser lors de la chirurgie de l'otospongiose.

INTRODUCTION: Stapes gusher means the leakage of perilymphatic liquid when opening the perilymphatic cistern. The perilymphatic liquid with a high pressure gushes with a great flow out of the cistern when the stapedotomy is executed. Otosclerosis surgery sometime brings to light abnormal contact between the inner ear and the sub-arachnoidian spaces in patients who didn't presented ear malformations. It's a very rare event (1/1000) which is different from a much more common and more moderate form of perilymphatic liquids high pressure (1/200). About 4 clinical observations, we compared our experience with other authors in specialist reviews. PURPOSE OF THE STUDY: About four observations, we confronted our experience with that of the literature. MATERIAL AND METHODS: Retrospective study between 1971 and 1998. It was about 3 males and 1 female, without antecedent except one of them who had been operated 5 years before for the opposite ear without gusher but without good audiometric result. They presented a conductive deafness with no answer of the stapedial reflex. We had 4 geysers during the platinotomia which were sealed with some connective tissue. RESULTS: Two patients had a post operative complete sensory hearing loss, one, a sensitive decline of the conduction thresholds (average 50 dB), the last one kept his bone conduction level with a mild sensory hearing loss. The most recent case had a scanner preoperatively which had not shown abnormality except for the focus of otosclerosis. DISCUSSION: Perilymphatic gusher is an unpredictable event that can not be diagnosed before the surgery, nether with clinical facts nor radiological elements. This involves serious consequences concerning not only the continuation of the surgical operation and the prognostic of the hearing but also concerning the danger of secondary meningeal infections. The best way to proceed in case of favourable cases consists in fitting the ossicular prothesis into the stapedotomy, when it's not to wide. Pieces of muscle can be used in some cases, taped on with biologic glu. Various techniques are used to lower the pression of the cerebrospinal liquid: hypertonic solutes, diuretic drugs, lumbar diversion. In all cases, it is necessary to start a wide spectrum antibiotic treatment and a vaccination against pneumococcis. CONCLUSION: The surgeon has to know all the option of the treatment when confronted with this situation in order to try to avoid tricky defect of the inner ear.