A Historical Perspective on Surgical Manipulation of the Membranous Labyrinth for Treatment of Meniere's Disease

Meniere's disease is an inner ear disorder without a known cause. Endolymphatic hydrops is a swelling of the endolymph spaces that has been observed consistently on post-mortem histology in patients with a history of Meniere's disease but can occur in asymptomatic individuals and in association with other diseases. Since its discovery, Meniere's disease has been a disorder managed primarily by otolaryngologists. Surgical treatments, therefore, have accompanied attempts at medical management. Inspired by patients' sensations of ear fullness and later by the histologic findings of hydrops, surgeons began manipulating the membranous labyrinth to relieve episodes of vertigo while attempting to preserve hearing. This review highlights this history of manipulation of the membranous labyrinth. These procedures indicate a rich history of innovation that parallels developments in otologic surgery. The studies involving patients are uniformly retrospective, with some procedures performed first in animal models of endolymphatic hydrops. Many approaches were endorsed by eminent otologic surgeons. Surgeries on the endolymphatic sac are performed by some surgeons today; however, procedures on the membranous labyrinth resulted in similar symptomatic relief through a minimally invasive technique, in many cases performed using only local anesthetic. Episodic vertigo in patients with Meniere's disease is a distressing symptom, yet spontaneous remissions are common. The reports of procedures on the membranous labyrinth reviewed here consistently indicated fewer vertigo episodes. Variable degrees of hearing loss were common following these procedures, and many were abandoned. Additional innovative surgeries are inevitable, but we must understand better the relationships among endolymphatic hydrops, Meniere's disease pathophysiology, and patient symptoms.

Incidence of Meniere disease in a tertiary healthcare center in Mumbai

<p><strong>Background:</strong> The disorder of the inner ear related to abnormally increased levels of endolymph in the membranous labyrinth is called Meniere’s disease. It is well studied that the prevalence of Meniere’s disease varies widely across regions. It was found to be 13% to 42% around the world in various places. It is important to know the incidence in India to know the population suffering and to plan better treatment strategies to cater to rehabilitation strategies. Hence, the current study was planned to estimate a load of Meniere’s disease in a tertiary care hospital in Mumbai.</p><p><strong>Methods:</strong> A retrospective analysis of the case reports was undertaken. As per American academy of otolaryngology-head and neck surgery (AAO-HNS, 2020), the patients were separated as definite and probable Meniere’s disease.</p><p><strong>Results:</strong> The descriptive analysis revealed that 6 in 1000 patients reporting for hearing evaluation in a tertiary care hospital are suffering from Meniere’s disease. There was a female preponderance found in the patients with a gender ratio of 1.85:1.</p><p><strong>Conclusions:</strong> The incidence of Meniere’s disease in a tertiary care hospital in Mumbai was found to be 0.61% of all the patients tested for hearing impairment.</p>

The ear

The delicate yet definitive deflections of the pinna (wing/fin) of the external ear contribute to the collection of sound. The external acoustic meatus is responsible for the transmission of sounds to the tympanic membrane, which in turn separates the external ear from the middle ear. The middle ear is an air filled (from the nasopharynx via the eustachian tube), mucous membrane lined space in the petrous temporal bone. It is separated from the inner ear by the medial wall of the tympanic cavity – bridged by the trio of ossicles. The inner ear refers to the bony and membranous labyrinth and their respective contents. The osseus labyrinth lies within the petrous temporal bone. It consists of the cochlea anteriorly, semicircular canals posterosuperiorly and intervening vestibule – the entrance hall to the inner ear whose lateral wall bears the oval window occupied by the stapes footplate.

Peculiarities of clinical manifestations of perilymphatic fistulas and evaluation of the effectiveness of treatment in patients with middle ear cholesteatoma

By the analysis of the 410 histories of illnesses of patients in 38 (9,26 %) are set perilymphatic fistula of different localization. Clinical, radiological intraoperative differences and going are set near the choice of method reconstructively operation depending on the type of perilymphatic fistula. After motion the extensive is certain more aggressive perilymphatic fistula is widespread, especially in the cases of cholesteatoma destruction of bone wall of labyrinth and simultaneous adhesion with a membranous labyrinth. On the stage of preoperative diagnostics from data of КТ of temporal bones of perilymphatic fistula set for 21 (55,3 %) patients and determined almost for all patients with extensive widespread perilymphatic fistula (in 12 patients from 13) and in 9 patients with limit perilymphatic fistula. By the preoperative inspection the positive test of fistula was determined only in 7 % patients. After localization more often perilymphatic fistula was determined at the level of lateral semicircular canal – for 23 patients (60,5 %). Plural localization of perilymphatic fistula with the united defeat of bone wall two and more semicircular canals educed 5 patients (13,1 %). The open variant of tympanoplasty is executed in 30 patients (78,9 %). The closed variant of tympanoplasty is in 6 (15,8 %) patients. Early exposure of perilymphatic fistula after the presence of not staggered endost and in good time reconstructive surgical interference is conducted with optimal combined by microscopically-endoscopic visualization for patients with chronic otitis media with cholesteatome will allow saving a rumor and will prevent development of irreversible complications from the side of internal ear.

Congenital malformations of the inner ear

Summary Congenital malformations of the inner ear consist of many different anomalies of the labyrinth. They often cause hearing loss, mostly of the sensorineural type. Eighty percent of hearing loss is caused by an anomaly of the membranous labyrinth, and 20% by an anomaly of the bone labyrinth. The role in pathogenesis is played by hereditary factors and influence of the environment. The treatment depends on the severity of the hearing loss, abnormalities of the external and middle ear, associated defects, and presence and function of the auditory nerve. We have modern hearing aids or implantable systems. Another options include a sign language and mouth-reading. The article includes a retrospective analysis of patients with congenital inner ear malformations at our tertiary center in 2010–2020. In conclusion, our patients clearly prove that even children with profound hearing loss are successfully implanted and restored hearing can be achieved in most of them. Key words congenital ear malformation – inner ear – hearing loss – anomalies of bony labyrinth – anomalies of membranous labyrinth – rehabilitation

508 The Early Use of Intratympanic Steroid Injections in the Treatment of Labyrinthitis Obliterans – A Case Report

Introduction Labyrinthitis Obliterans is an inner ear condition characterised by pathological sclerosis and ossification of the membranous labyrinth as a response to inflammatory insult. It is associated with profound sensorineural hearing loss and occasionally dizziness and/or vertigo.In this case report, we describe the use of intratympanic steroid injections in a patient presenting with sudden sensorineural hearing loss (SSNHL) with a radiological diagnosis of Labyrinthitis Obliterans. Whilst many studies suggest the role of oral steroids in SSNHL, there is no current evidence regarding the role of intratympanic steroid injections specifically for the treatment of this condition. Case Presentation A 60-year-old gentleman was referred to ENT with a 3-day history of sudden right sided hearing loss. There was no improvement in his Pure Tone Audiometry (PTA) results after 7 days of oral Prednisolone. Subsequently, an MRI Internal Acoustic Meatus showed evidence of right sided Labyrinthitis Obliterans. The patient was initiated on a course of intratympanic Methylprednisolone injections 10 days after initial onset of symptoms. A significant improvement in his baseline hearing of over 60dB was demonstrated after 4 courses of injections over a 3-month period. Discussion Our findings reveal that despite a diagnosis of Labyrinthitis Obliterans, the early use of intratympanic steroid injections can significantly improve hearing threshold and quality of life of the patient. The biggest hurdle in the management of Labyrinthitis Obliterans is establishing the diagnosis early and ensuring timely treatment, therefore we would advocate the early use of MRI and early intervention with intratympanic steroid injections.

MR Imaging of Cochlear Modiolus and Endolymphatic Hydrops in Patients With Menière's Disease

Background: Menière's disease (MD) is an inner ear disorder characterized by recurrent episodes of spontaneous vertigo, unilateral low-frequency sensorineural hearing loss, tinnitus, and aural fullness. Current diagnosis still often has to rely on subjective and audiometric criteria only, although endolymphatic hydrops is recognized as the pathophysiological substrate of the disease, having been demonstrated in anatomical pathological studies and by magnetic resonance (MRI). The modiolus has a close functional and anatomical relationship with the cochlear nerve and membranous labyrinth and can be evaluated with MRI but no data exist on the modiolar size in MD.Purpose: Our purpose is to examine the following hypothesis. Is cochlear modiolus smaller in symptomatic ears in MD?Methods: We used a retrospective 3 Tesla MR study (heavily T2-weighted 3D fast asymmetric spin-echo images and 0.5 mm slice thickness) comparing the mean modiolar area (MMA) in the index and best ears of eight patients with definite MD based on audiometric data. The obtained MMA values were compared against the audiometric data and the presence of vestibular endolymphatic hydrops.Results: No differences were seen in MMA between best and worst ears. Ears with a pure tone average (PTA) ≥25 dB and more pronounced endolymphatic hydrops showed lower MMA (not statistically significant). Two patients with extreme endolymphatic hydrops showed a noteworthy ipsilateral decrease in the cochlear modiolus area.Conclusion: No differences were seen in MMA between best and worst ears in definite MD. Worse hearing function (PTA ≥ 25dB) and more pronounced endolymphatic hydrops seem to be associated with lower MMA. This might be related to bone remodeling as a consequence of endolymphatic hydrops. Further research is needed to corroborate and explore these findings.

Present and future of the labyrinth imaging: Focus on the use of T2-weighted and contrast-enhanced delayed FLAIR (1 h) sequences

OBJECTIVE: Part of the recent progress in the labyrinth imaging has been made possible by the rise of contrast-free T2-weighted and delayed (1h) FLAIR sequences. The aim of this article is to review evidence for the use of these two sequences to image the inner ear, especially the posterior membranous labyrinth. MATERIAL AND METHODS: We analyzed MRI-based papers (2007–2020)using high-resolution T2-weighted or contrast-enhanced FLAIR (1h) sequences to image the inner ear. RESULTS: T2-weighted sequences (3T MRI)enabled the visualization of the posterior membranous labyrinth with good correlation when compared to corresponding histological slices.Significant progress has been made, especially in terms of scanning time, aiming at reducing it, in order to decrease motions artifacts. The saccule is visible on a 3T MRI without significant motion artifacts. Its shape is ovoid, with a maximum height and width of 1.6 and 1.4 mm, respectively. An enlarged saccule was observed in 84%of patients with unilateral Meniere’s disease, in 28%of patients with vestibular schwannomas (VS) and 47%of patients with intralabyrinthine schwannomas. VS obstructing the internal auditory canal caused a decrease of the perilymphatic signal (more moderate decrease in meningiomas) on T2 gradient-echo images. Contrast-enhanced FLAIR sequences are useful to image vestibular/facial neuritis and inflammatory inner ear diseases. CONCLUSION: Precise analysis of the posterior membranous labyrinth, in terms of size, shape and signal intensity, is possible on a 3T MRI using high-resolution gradient-echo T2-weighted sequences. Such sequences are an interesting add-on to delayed (4h30) FLAIR-based protocols for labyrinth imaging.

“Sandwich technique” enables preservation of hearing and antivertiginous effect in cholesteatomatous labyrinthine fistula

Purpose The aim of the study was to assess hearing, surgical and clinical results of the treatment in patients with cholesteatomatous labyrinthine fistula (LF) focusing on the different techniques and materials used in the management. Methods Study group included 465 patients. Cases with LFs discovered or confirmed during surgical procedure were thoroughly analyzed. Results LFs were noted in 11.4% of all cases. Thirty-eight patients, with all follow-up data available, were included into the further analysis. Most LFs were located in the lateral semicircular canal (87%). LFs were assessed as small in 2 cases, as medium in 24 patients while 12 were described as large. Based on Dornhoffer and Milewski classification, 50% of LFs were classified as IIa, 24% as IIb, 6 LFs were very deep (type III), while 4—superficial (type I). The size and type of LF did not influence postsurgical complaints (p = 0.1070, p = 0.3187, respectively). Vertigo was less frequent in LFs treated by “sandwich technique”, especially those with opened endosteum. In 30 (79%) patients, hearing improved or did not change after surgery. Hearing outcomes were significantly better in the ears operated by means of CWU technique (p = 0.0339), in LFs with intact membranous labyrinth (p = 0.0139) and when “sandwich technique” was performed (p = 0.0159). Postsurgical bone conduction thresholds levels were significantly better in LFs covered by “sandwich method” (p = 0.0440). Conclusion “Sandwich technique” (temporal fascia–bone pate–temporal fascia) enables preservation of hearing as well as antivertiginous effect in patients with cholesteatomatous labyrinthine fistula.