Computed tomographic 3D analysis of the cochlear aqueduct-potential and limitations of clinical imaging.

Background: The cochlear aqueduct (CA), which connects the scala tympani and the subarachnoid space, and its accompanying structures appear to have a significant relevance during cochlear implantation and an accurate visualization in clinical imaging is of great interest. Aims and Objective: This study aims to determine which potential and limitations clinically available imaging modalities have in the visualization of the CA. Methods: Micro-CT, flat-panel volume computed tomography with and without secondary reconstruction (fpVCT, fpVCTseco) and multislice computed tomography (MSCT) of 10 temporal bone specimen were used for 3D analysis of the CA. Results: FpVCTseco proved superior in visualizing the associated structures and lateral portions of the CA, which merge into the basal turn of the cochlea. All clinical imaging modalities proved equal in analyzing the length, total volume of the CA and its area of the medial orifice. Conclusion: The choice of the most accurate clinical imaging modality to evaluate the CA and its associated structures depends on the clinical or scientific question. Furthermore, this study should provide a basis for further investigations analyzing the CA.

Anatomy of the Round Window Region With Relation to Selection of Entry Site Into the Scala Tympani.

OBJECTIVES/HYPOTHESIS: The aim of cochlear implantation is to safely insert an electrode array into the scala tympani (ST) while avoiding damage to surrounding structures. There is disagreement on the optimal way of entering the ST-the round window (RW) approach versus cochleostomy. Regardless of the chosen approach, it is vital to understand the regional anatomy, which is complex, difficult to conceptualize, and rarely dissected in temporal bone courses. The goal of this study was to examine the anatomy of the RW to gain more in-depth knowledge on the local relationships of the anatomical structures and propose an approach for entering the ST in cochlear implant surgery tailored to the encountered anatomy. STUDY DESIGN: Cadaveric prevalence study and expert opinion with literature review. METHODS: Cadaveric temporal bone dissection (n = 13) by the first author assessing the RW anatomy. RESULTS: The round window membrane (RWM) and the osseous spiral lamina (OSL) are curved structures, each with a horizontal and a vertical part. The two horizontal portions are very closely apposed. The relationship between the OSL and the RWM determines the best site for a cochleostomy, which if required is best placed anteroinferiorly to the RWM. The distance between the oval window inferior margin and the RW membrane is less than 2 to 3 mm. The ST initially extends inferiorly and medially to the RW. CONCLUSIONS: The findings of our dissection have implications for cochlear implant surgery in aiming to avoid trauma to the OSL and basilar membrane and aid decision making in choosing the safest surgical approach. LEVEL OF EVIDENCE: 5. Laryngoscope, 131:E598-E604, 2021.

Radiologic Evaluation of the Round Window Niche Configuration: A One-Stop Approach.

BACKGROUND: Preoperative radiological prediction of the round window niche configuration. METHODS: Fifty patients were evaluated. A single-axial high-resolution computed tomography image at the level of the cochlear aqueduct was compared to the intraoperative surgical images. Radiological configuration was classified as open, hooded, or covered depending on the extent of bony overhang. Surgical images were processed using Image J software to determine the amount of drilling required before the round window membrane is exposed. These images were classified according to the St. Thomas classification into 3 grades. RESULTS: In all patients, the axial cut showing the cochlear aqueduct was obtained. There were 12 cases in the open category, 17 in the hooded category, and 21 in the covered one. Intraoperatively, the actual findings were type I 12, type II 18, and type III 20. The correspondence between the expected and actual classification was correct in 8, 12, and 18 cases, respectively. Comparing the intraoperative findings with the expected radiological configuration, there was a good concordance with a statistically non-significant difference ( χ2=0.2613; P=.87751). CONCLUSION: It is possible to predict the configuration of the round window niche on a single-axial computed tomography cut and plan the most suitable axis of approach and predict the amount of drilling expected to expose the round window membrane.

Atraumatic Scala Tympani Cochleostomy; Resolution of the Dilemma.

OBJECTIVES: While an accurate placement in cochleostomy is critical to ensure appropriate insertion of the cochlear implant (CI) electrode into the scala tympani (ST), the choice of preferred cochleostomy sites widely varied among experienced surgeons. We present a novel technique for precise yet readily applicable localization of the optimum site for performing ST cochleostomy. MATERIAL AND METHODS: Twenty fresh frozen temporal bones were dissected using the mastoidectomy-posterior tympanotomy approach. Based on the facial nerve and the margins of the round window membrane (RWM), the cochleostomy site was chosen to insert the electrode into the ST while preserving the surrounding intracochlear structures. RESULTS: There is a limited safe area suitable for the ST implantation in the area inferior and anterior to the RWM. There is a higher risk of scala vestibuli (SV) insertion anterior to that area. Posterior to that area, the cochlear aqueduct (CA) and inferior cochlear vein (ICV) are liable for the injury. CONCLUSION: For atraumatic CI, precise and easy localization of the site of cochleostomy play a pivotal role in preserving intracochlear structures. Accurate setting of the vertical and horizontal orientations is mandatory before choosing the site of cochleostomy. The facial nerve and the margins of the RWM offer a very helpful clue for such localization; meanwhile, it is readily identifiable in the surgical field.

Sneezing and Perilymphatic Fistula of the Round Window: Case Report and Systematic Review of the Literature.

The goal of the present study was to describe a case of perilymphatic fistula (PLF) of the round window (RW) that occurred after sneezing, along with a review of the literature. We report a case of PLF of RW, which was provoked by sneezing, and its consequent medical and surgical treatments. With respect to the review of the literature, articles were initially selected based on their titles or abstracts, followed by methodological evaluation. The patient underwent an explorative tympanotomy (ET) with packaging of RW with the pericondrium, following which the patient's complaints regarding vertigo and imbalance disappeared, but the severe sensorineural hearing loss persisted. For the literature review, five references were selected. These studies showed a great variety in the clinical presentation and healing of symptoms. Sneezing represents a rare but well-recognized cause of PLF, as reported in our case. The correct selection of patients who should undergo ET and an early surgical repair of PLF are mandatory for better outcomes, especially in case of hearing.

The role of explorative tympanotomy in patients with sudden sensorineural hearing loss with and without perilymphatic fistula.

PURPOSE: The purpose of this study was to describe the role of explorative tympanotomy in patients with Profound Sudden Sensorineural Hearing Loss (SSNHL) without clinical evidence of perilymphatic or labyrinthine fistula and to compare intraoperative findings with the postoperative hearing outcome. STUDY DESIGN: Retrospective study of all patients diagnosed with SSNHL who underwent explorative tympanotomy between 2002 and 2005. SETTINGS: Tertiary care university-affiliated hospital. SUBJECTS AND METHODS: Eighty-two patients were diagnosed with unilateral profound SSNHL and underwent tympanotomy with sealing of the round and oval windows. Values of pure tone audiograms and percentage hearing loss of patients with and without intraoperative diagnosed perilymphatic fistula (PLF) were compared and analyzed. RESULTS: PLF was diagnosed in 28% cases intraoperatively. In most cases, hearing improved significantly after surgery. Interestingly, patients with PLF had a 2.4 times greater decrease of percentage hearing loss compared to patients without PLF. CONCLUSIONS: Explorative tympanotomy seems to be useful in patients with profound SSNHL. Patients with PLF benefit more from the surgical procedure and have better outcome than patients without PLF.

Perilymph fistula: the patients' experience.

OBJECTIVE: This study aimed to assess the experiences and outcomes of patients who underwent surgical repair of a perilymph fistula in Norfolk, UK. METHODS: The study involved a retrospective questionnaire-based patient survey and case note review of patients who had undergone tympanotomy and perilymph fistula repair between 1998 and 2012 in two district general hospitals. RESULTS: Fourteen patients underwent 20 procedures, of whom 7 completed the pre- and post-operative Vertigo Symptom Scale. In five patients, there was no obvious precipitating cause. Perilymph fistula was precipitated by noise in one patient, by a pressure-increasing event in six patients and by trauma in two patients. The Vertigo Symptom Scale scores showed a statistically significant improvement following surgical repair, from a median of 67 (out of 175) pre-operatively to 19 post-operatively. CONCLUSION: In selected patients with vertigo, perilymph fistula should be considered; surgical repair can significantly improve symptoms.

X-ray microtomography study of otic capsule deficiencies: three-dimensional modelling of the fissula ante fenestram.

BACKGROUND: The postulated sites of perilymph fistulae involve otic capsule deficiencies, in particular, at the fissula ante fenestram. Histological studies have revealed this to be a channel extending from the middle ear, and becoming continuous with the inner ear medial to the anterior limit of the oval window. The relationship between a patent fissula and symptoms of perilymph fistula is contentious. OBJECTIVE: The understanding of the anatomy of the fissula ante fenestram is incomplete. Histopathology is inherently destructive to the delicate ultrastructure of the middle and inner ear. Conversely, X-ray microtomography allows non-destructive examination of the otic capsule. In this study, we used X-ray microtomography to characterise the fissula ante fenestram. MATERIALS AND METHODS: We imaged cadaveric temporal bones with X-ray microtomography. We used the Avizo Fire (Visualization Science Group, Merignac Cedex, France) software to perform post-processing and image analysis. RESULTS: Three-dimensional modelling of the fissula ante fenestram allowed stratification into four forms: rudimentary pit; partial fissula; complete occluded fissula; and complete patent fissula. CONCLUSION: X-ray microtomography showed that the fissula ante fenestram is present in various forms from rudimentary pit to complete deficiency of the otic capsule. This understanding may have implications for otologic surgery and clinical diagnosis of perilymph fistula.

Congenital perilymph fistula causing recurrent meningitis: lessons learnt from a single-institution case series.

OBJECTIVE: To study the steps involved in definitive evaluation and successful management of patients with congenital perilymph fistula presenting with recurrent meningitis. STUDY DESIGN: Case series with chart review. SETTING: Tertiary care center. SUBJECTS AND METHODS: The case records of 11 patients (12 ears) treated for congenital perilymph fistula presenting with recurrent meningitis were reviewed to ascertain their clinical, radiological, and intraoperative features and outcome following surgery. RESULTS: Most patients presented after at least 3 episodes of meningitis (range, 2-10 episodes). Ipsilateral hearing loss was present in 9 of 12 ears, with normal hearing in 3 patients. High-resolution computed tomography and/or magnetic resonance imaging scanning of the temporal bone contributed to the diagnosis in 75% of cases but was normal in 3 cases (25%). Oval window and round window defects were the most common (66.7% and 63.6%, respectively). Four ears (33.3%) had more than 1 defect. The unusual presentations included 2 patients who presented in adulthood, a patient with a defect in the medial wall of the attic, and 3 patients with normal radiological findings. Follow-up ranged from 1 to 11 years (median, 2 years). There were 2 failures following simple fistula closure with cessation of symptoms after vestibular obliteration. No patient was readmitted with recurrent meningitis after definitive surgery. CONCLUSION: Up to 25% of patients with recurrent meningitis secondary to congenital perilymph fistula may have normal audiological and radiological assessment necessitating exploratory tympanotomy. Vestibular obliteration, rather than simple fistula closure, prevents recurrence.

Enlarged cochlear aqueducts: a potential route for CSF gushers in patients with enlarged vestibular aqueducts.

OBJECTIVE: We investigated whether the size of the cochlear aqueduct (CA) is increased in patients with enlarged vestibular aqueducts (EVAs) compared with individuals with normal inner ear anatomy. Furthermore, we assessed whether the size of the CA is related to the cerebrospinal fluid (CSF) gusher during cochlear implantation (CI) surgery. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. PATIENTS: The medical records for 35 patients with a biallelic SLC26A4 mutation were reviewed. All of the patients were confirmed to have EVA by temporal bone computed tomography (TBCT) and biallelic SLC26A4 mutation by genetic analysis. Thirty-six age- and sex-matched patients without a mutation in the SLC26A4 gene were selected as comparison group. MAIN OUTCOME MEASURES: The diameters of the CA and vestibular aqueduct (VA) were measured, and the type of CA was evaluated using TBCT. RESULTS: The CA was larger in patients with EVA (2.39 ± 0.99 mm) as compared with controls (1.76 ± 0.87 mm; p < 0.001). The types that could be most easily visualized from the subarachnoid space to the cochlea were more common in patients with EVA. Furthermore, mean CA size was significantly larger in EVA patients with CSF gushers (3.65 ± 1.12 mm) as compared with those without CSF gushers (2.03 ± 0.66 mm; p < 0.001). CONCLUSION: The CA is a potential pathway for CSF gushers between the subarachnoid space and the inner ear. Therefore, we suggest that evaluation of the CA by TBCT may be helpful in predicting intraoperative CSF gushers in patients with EVA.

Late pneumolabyrinth after undiagnosed post-traumatic perilymphatic fistula. Case report illustrating the importance of systematic emergency management.

INTRODUCTION: Temporal bone fracture is a common complication of high-energy cranial trauma. Labyrinth involvement is rare, but there is a risk of perilymphatic rupture that is often underestimated on initial clinical examination due to the predominance of neurological and/or somatic symptoms. CASE REPORT: A patient presented with overlooked perilymphatic fistula, decompensated by hyperpressure effort due to poorly adapted management. DISCUSSION: Following a review of the literature on post-traumatic pneumolabyrinth, overall management (from diagnosis to treatment, via prevention advice) was analyzed. A constructive critique of the patient's treatment was thus made. CONCLUSION: We argue for a systematic management protocol in cranial trauma with temporal bone fracture, comprising ENT examination, millimetric-scale cross-sectional imaging of the fracture site, and standardized counseling to prevent late complications.

Noise-induced perilymph fistula.

Perilymph fistulae are difficult to diagnose because they present with a wide variety of signs and symptoms, they are associated with many etiologies, and they often mimic other conditions. In this article, we describe a case of perilymph fistula that featured one of its more rare causes: acoustic trauma--specifically, damage from a loud blast from the siren of a fire engine. We also review the literature and discuss the difficulties of diagnosis and treatment and the possible mechanisms by which acoustic trauma and other etiologies cause perilymph fistulae.

Could vestibular evoked myogenic potentials (VEMPs) also be useful in the diagnosis of perilymphatic fistula?

The role of vestibular evoked myogenic potentials (VEMPs) is at this time indisputable in the study of vestibular disorders. Furthermore, VEMPs are widely accepted as a diagnostic tool when a superior semicircular canal dehiscence (SCD) is suspected, presenting in such cases a lowering of threshold values able to raise a recordable response due to increased inner ear immittance. According to the same principle, the possibility of another kind of alteration having the same effect on the inner ear might be considered when high-resolution computed tomography has excluded the presence of an SCD. In this paper four cases are described in which high-resolution computed tomography showed normal features without any labyrinthine dehiscence and VEMP threshold values were lowered; the appropriateness of suspecting a perilymphatic fistula in such cases and resorting to VEMPs in detecting a perilymphatic fistula is discussed.

Anatomic landmarks of the glossopharyngeal nerve: a microsurgical anatomic study.

OBJECTIVE: Compared with other lower cranial nerves, the glossopharyngeal nerve (GPhN) is well hidden within the jugular foramen, at the infratemporal fossa, and in the deep layers of the neck. This study aims to disclose the course of the GPhN and point out landmarks to aid in its exposure. METHODS: The GPhN was studied in 10 cadaveric heads (20 sides) injected with colored latex for microsurgical dissection. The specimens were dissected under the surgical microscope. RESULTS: The GPhN can be divided into three portions: cisternal, jugular foramen, and extracranial. The rootlets of the GPhN emerge from the postolivary sulcus and course ventral to the flocculus and choroid plexus of the lateral recess of the fourth ventricle. The nerve then enters the jugular foramen through the uppermost porus (pars nervosa) and is separated from the vagus and accessory nerves by a fibrous crest. The cochlear aqueduct opens to the roof of this porus. On four sides in the cadaver specimens (20%), the GPhN traversed a separate bony canal within the jugular foramen; no separate canal was found in the other cadavers. In all specimens, the Jacobson's (tympanic) nerve emerged from the inferior ganglion of the GPhN, and the Arnold's (auricular branch of the vagus) nerve also consisted of branches from the GPhN. The GPhN exits from the jugular foramen posteromedial to the styloid process and the styloid muscles. The last four cranial nerves and the internal jugular vein pass through a narrow space between the transverse process of the atlas (C1) and the styloid process. The styloid muscles are a pyramid shape, the tip of which is formed by the attachment of the styloid muscles to the styloid process. The GPhN crosses to the anterior side of the stylopharyngeus muscle at the junction of the stylopharyngeus, middle constrictor, and hyoglossal muscles, which are at the base of the pyramid. The middle constrictor muscle forms a wall between the GPhN and the hypoglossal nerve in this region. Then, the GPhN gives off a lingual branch and deepens to innervate the pharyngeal mucosa. CONCLUSION: Two landmarks help to identify the GPhN in the subarachnoid space: the choroid plexus of the lateral recess of the fourth ventricle and the dural entrance porus of the jugular foramen. The opening of the cochlear aqueduct, the mastoid canaliculus, and the inferior tympanic canaliculus are three landmarks of the GPhN within the jugular foramen. Finally, the base of the styloid process, the base of the styloid pyramid, and the transverse process of the atlas serve as three landmarks of the GPhN at the extracranial region in the infratemporal fossa.

[Inner ear and subarachnoid spaces: relations and diseases]. / Oreille interne et espaces sous-arachnoïdiens.

The purpose of this article is to describe, with 5 clinical cases, the physiological communications between the inner ear and the subarachnoid spaces (SAS) and present the imaging features with regard to. Therefore we briefly illustrate abnormal communications between SAS and perilymphatic fluids in certain cochlear and internal acoustic meatus (IAM) malformations and their consequences. Imaging features may depict diffusion pathway of bacterial meningitis to membranous labyrinth via the cochlear aqueduct or via the IAM. Rarely, in some patients referred for cochleovestibular symptoms, imaging features may display skull base tumors involving the area of cochlear or vestibular aqueduct aperture. Therefore, in patients referred for cochleovestibular symptoms, MR and CT study should carefully scrutinise not only the IAM but also the aperture of the cochlear and the vestibular aqueducts and the cerebellopontine meninges.

[Rupture of the round window--detection with fluorescence endoscopy]. / Rundfensterruptur--Nachweis mittels Fluoreszenzendoskopie.

Rupture of the round window membrane as a special cause of inner ear deafness is widely accepted after changing pressure levels, e.g. in diving. However, even without a barotrauma before, the spontaneous rupture of the round window membrane is suspected occasionally in patients with sudden hearing loss and/or vertigo and tinnitus. To carry through the tympanotomy is decided by ENT surgeons often in cases of progressive hearing loss despite infusion therapy. Perilymph fistulas have been detected relatively seldom, compared to the number of reported operations by several authors. However, covering the round niche with connective tissue leads to the improvement of symptoms sometimes even in cases without microscopical evidence of fistula. Within the last 3 years 14 patients suffering sudden hearing loss of one ear underwent tympanotomy in our department. Of these patients 8 reached restitution of the hearing ability. Especially 2 patients with sudden deafness caused by spontaneous rupture of the round window membrane are reported in the following article. Perilymph fistulas were detected in these cases by IV-application of fluorescein and fluorescence endoscopy of the middle ear. Both patients obtained a normal hearing curve within 1 week after surgical intervention and obliteration of the round niche.

Syndrome of recurrent meninigitis due to congenital perilymph fistula with two different clinical presentations.

Recurrent meningitis secondary to a congenital labyrinthine anomaly is a rare clinical entity, diagnosis of which is dependent upon certain clinical, radiological and intraoperative features. In the following report we describe two children with congenital labyrinthine fistula and recurrent meningitis whose clinical presentation, radiological features and intraoperative findings were dissimilar and thus, illustrative of two different ways of presentation of this rare disorder. While one had a classical Mondini defect and unilateral hearing loss, the other had normal audiometric and radiographic findings. The fistulae were successfully closed via a tympanotomy approach in both the patients.