scholarly journals A gyógyszeres kezelésre nem javuló hirtelen halláscsökkenés lépcsőzetes sebészi terápiája

2021 ◽  
Vol 162 (51) ◽  
pp. 2055-2060
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Early Stage ◽  
Round Window ◽  
Complete Recovery ◽  
Round Window Membrane ◽  
Systemic Steroid ◽  
Profound Hearing Loss

Összefoglaló. A hirtelen halláscsökkenés patofiziológiája még nagyrészt tisztázatlan, így oki terápia nem lehetséges. Az elsődleges kezelést a helyileg vagy szisztémásan adott kortikoszteroid jelenti, egységes protokoll azonban nem áll rendelkezésre. Nagy vagy súlyos fokú hirtelen halláscsökkenés esetén kóroki tényezőként felmerül a perilymphafistula lehetősége még azoknál a betegeknél is, akiknél nem szerepel trauma az anamnézisben. A kórkép műtéti kezelése a dobüreg feltárását követően a belső fül ablakainak obliterálása. Amennyiben ez a megoldás nem eredményez megfelelő hallásjavulást, hagyományos vagy implantálható hallókészülékek alkalmazása javasolt. A közleményben részletezett esetünkben teljes siketséggel járó, jobb oldali hirtelen halláscsökkenés alakult ki, melynek hátterében egyértelmű okot azonosítani nem sikerült. Az eredménytelen kombinált, intratympanalis és szisztémás szteroidkezelést követően exploratív tympanotomiát végeztünk, melynek során a belső fül ablakait obliteráltuk. Hallásjavulást ezt követően sem sikerült kimutatni, így cochlearis implantáció elvégzése mellett döntöttünk. Az implantációt a kerek ablakon keresztül végeztük, mely alapján kijelenthetjük, hogy az előzetes kerekablak-obliteráció nem zárja ki a későbbi cochlearis implantációt. Orv Hetil. 2021; 162(51): 2055–2060. Summary. The pathophysiology of sudden sensorineural hearing loss is mainly unknown, therefore no causative treatment exists. Systemic and local administration of corticosteroids serves as first line therapy although protocols vary. In cases of severe or profound hearing loss with no improvement for medical therapy, perilymphatic fistulae can be assumed even without any history of trauma. Therefore, inner ear window obliteration as a primary surgical option in the early stage can be considered. For patients without complete recovery, conventional hearing aids or implantable hearing devices can be offered. In our case report, we present a patient with right sided idiopathic sudden deafness. After failure of conservative combined intratympanic and systemic steroid therapy, explorative tympanotomy and obliteration of the inner ear windows were performed. As no hearing improvement was witnessed, successful cochlear implantation via round window insertion was performed. Our case justifies that obliterating the round window membrane does not rule out further successful cochlear implantation. Orv Hetil. 2021; 162(51): 2055–2060.

scholarly journals A Window of Opportunity: Perilymph Sampling from the Round Window Membrane Can Advance Inner Ear Diagnostics and Therapeutics

2022 ◽  
Vol 11 (2) ◽  
pp. 316
Author(s):  
Madeleine St. Peter ◽  
Athanasia Warnecke ◽  
Hinrich Staecker
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Inner Ear ◽  
Hearing Aids ◽  
Biomarker Discovery ◽  
Treatment Options ◽  
Round Window ◽  
Sensorineural Hearing ◽  
Round Window Membrane ◽  
Promising Source

In the clinical setting, the pathophysiology of sensorineural hearing loss is poorly defined and there are currently no diagnostic tests available to differentiate between subtypes. This often leaves patients with generalized treatment options such as steroids, hearing aids, or cochlear implantation. The gold standard for localizing disease is direct biopsy or imaging of the affected tissue; however, the inaccessibility and fragility of the cochlea make these techniques difficult. Thus, the establishment of an indirect biopsy, a sampling of inner fluids, is needed to advance inner ear diagnostics and allow for the development of novel therapeutics for inner ear disease. A promising source is perilymph, an inner ear liquid that bathes multiple structures critical to sound transduction. Intraoperative perilymph sampling via the round window membrane of the cochlea has been successfully used to profile the proteome, metabolome, and transcriptome of the inner ear and is a potential source of biomarker discovery. Despite its potential to provide insight into inner ear pathologies, human perilymph sampling continues to be controversial and is currently performed only in conjunction with a planned procedure where the inner ear is opened. Here, we review the safety of procedures in which the inner ear is opened, highlight studies where perilymph analysis has advanced our knowledge of inner ear diseases, and finally propose that perilymph sampling could be done as a stand-alone procedure, thereby advancing our ability to accurately classify sensorineural hearing loss.

scholarly journals Cochlear Implantation Following Explorative Tympanotomy in Patients With Sudden Sensorineural Hearing Loss: Surgical Features and Audiological Outcomes

2021 ◽  
pp. 014556132110091
Author(s):  
Robin Rupp ◽  
Joachim Hornung ◽  
Matthias Balk ◽  
Matti Sievert ◽  
Sarina Müller ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Connective Tissue ◽  
Sensorineural Hearing Loss ◽  
Cochlear Implantation ◽  
Round Window ◽  
Sudden Sensorineural Hearing Loss ◽  
Sensorineural Hearing ◽  
Round Window Membrane ◽  
Hearing Results ◽  
Round Window Niche

Objective: To investigate the anatomical status of the round window niche and hearing outcome of cochlear implantation (CI) after explorative tympanotomy (ExT) with sealing of the round window membrane in patients with sudden sensorineural hearing loss at a tertiary referral medical center. Methods: Between January 1, 2007, and July 30, 2020, 1602 patients underwent CI at our department. Out of these, all patients previously treated by ExT with sealing of the round window membrane because of unilateral sudden hearing loss were included in the study. A retrospective chart review was conducted concerning method of round window membrane sealing, intraoperative findings during CI, postoperative imaging, and hearing results. Results: Twenty one patients (9 females; 8 right ears; 54.3 years [± 12.9 years]) underwent ExT with sealing of the round window membrane with subsequent CI after 26.6 months (± 32.9 mo) on average. During CI, in 76% of cases (n = 16), the round window niche was blocked by connective tissue due to the previous intervention but could be removed completely in all cases. The connective tissue itself and its removal had no detrimental effects on the round window membrane. Postoperative computed tomography scan showed no electrode dislocation. Mean postoperative word recognition score after 3 months was 57.4% (± 17.2%) and improved significantly to 73.1% (± 16.4%, P = .005) after 2 years. Conclusion: Performing CI after preceding ExT, connective tissue has to be expected blocking the round window niche. Remaining tissue can be removed safely and does not alter the round window membrane allowing for a proper electrode insertion. Short- and long-term hearing results are satisfactory. Consequently, ExT with sealing of the round window membrane in patients with sudden sensorineural hearing loss does not impede subsequent CI that can still be performed safely.

Direct drug application to the round window: A comparative study of ototoxicity in rats

2009 ◽  
Vol 141 (5) ◽  
pp. 584-590 ◽  
Author(s):  
Silvia Murillo-Cuesta ◽  
Fernando García-Alcántara ◽  
Elena Vacas ◽  
Jon Alexander Sistiaga ◽  
Guadalupe Camarero ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Cell Damage ◽  
Round Window ◽  
Drug Application ◽  
Local Drug Delivery ◽  
Saline Control ◽  
Round Window Membrane ◽  
Cochlear Function ◽  
Profound Hearing Loss ◽  
Brainstem Response

Objective: To assess the validity of inducing ototoxicity in rats by applying a sponge soaked in kanamycin and furosemide on the round window. Study Design: Basic, randomized, nonblind experimental study. Setting: Animal models of cochlear damage and reliable methods of local drug delivery are fundamental to study hearing loss and to design new therapies. Subjects and Methods: Four experimental groups of six Wistar rats with different methods of drug administration were used: (1) injection of subcutaneous kanamycin (400 mg/kg) and intravenous furosemide (100 mg/kg); (2) local application of a sponge soaked in saline close to the round window; (3) animals for which the sponge was soaked in a solution containing kanamycin (200 mg/mL) and furosemide (50 mg/mL); and (4) sham-operated rats. The tympanic bulla was exposed using a ventral approach, and a bullostomy was performed to visualize the round window membrane. Cochlear function was assessed by measuring the auditory brainstem response, and hearing thresholds in response to click and tone burst stimuli were determined as peak and interpeak latencies. At the end of the study, cochlear histology was analyzed. Results: Systemic administration of kanamycin and furosemide induced profound hearing loss and severe hair cell damage. Local delivery of these ototoxic drugs caused comparable damage but avoided the systemic side effects of the drug. Sham-operated and saline control animals did not experience functional alterations. Conclusion: Situating a sponge soaked in kanamycin and furosemide on the round window membrane through the ventral approach is a reliable method to provoke local ototoxicity in rats.

scholarly journals Preoperative Evaluation and Surgical Outcome of Cochlear Implantation in NIENT

2020 ◽  
Vol 26 (2) ◽  
pp. 128-135
Author(s):  
Md Zakaria Sarker ◽  
Muhammad Rafiqul Islam ◽  
Utpal Kumar Dutta ◽  
Debabrota Roy ◽  
AHM Ferdows Nur ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Facial Nerve ◽  
Psychosocial Development ◽  
Cochlear Implantation ◽  
Preoperative Evaluation ◽  
Round Window ◽  
Round Window Membrane ◽  
Safe Procedure ◽  
Female Ratio ◽  
Facial Recess

Background: Sensory neural hearing loss (SNHL) is the most common congenital sensory deficit, with an incidence of one to three per 1000 live births. Acoustic deprivation during the first 3 years of life can hinder speech and language acquisition with significant negative consequences on a child’s educational and psychosocial development. The gold standard intervention for permanent severe to profound hearing loss is cochlear implantation. Cochlear implant (CI); is a semi implantable electronic device that bypass the cochlea. Objectives: An observational study was carried out on 40 cases of pre-lingual deaf to find out the causes of pre-lingual deaf, to evaluate the preoperative procedures to set ideal criteria for pre -lingual cochlear implantation and to evaluate surgical procedure and outcome of cochlear implantations. Methods: Evaluation of the candidates included patient medical history, general health checkup, ENT examination, audiometric evaluation, CT and MRI scans, psychological profile of the candidate. A limited cortical mastoidectomy was performed. The facial recess was opened using the fossa of incudis as an initial landmark. The round window niche was visualized through the facial recess about 2 mm inferior to the stapes. A cochleostomy created by drilling over the basal turn of the cochlea anterior and inferior to the annulus of the round window membrane. The electrode array was then carefully inserted through the fenestra into the scala tympani of the cochlea. Electrophysiological testing (Neural Response Telemetry: NRT) was performed to verify the correct placement of active electrodes. Resulst: Among them 22 (55%) were male and 18 (45%) were female. Male female ratio was 1.2:1. Age distribution at implantation was 3.3±1.054(SD). Average hearing loss was 96.4±5.3(SD) dB and in aided audiogram was 63.7±4.6(SD) dB. Overall complications occurred in 10 (25%) cases. Transient facial nerve paresis in 2 (5%), injury of tympanic membrane in 1(2.5%), seroma 4(10%) and delayed otitis media in 1(2.5%) were observed. Major postoperative complications occurred in 2 cases including facial nerve palsy in 1(2.5%) case and spontaneous device failure in 1(2.5%) case. Conclusion: The result of this survey was find out the risk factor of congenital hearing loss. With a thorough preoperative evaluation, we can select proper candidates for CI which is a reliable and safe procedure with a low percentage of severe complication. Bangladesh J Otorhinolaryngol; October 2020; 26(2): 128-135

scholarly journals Efficiency of a Dexamethasone Nanosuspension as an Intratympanic Injection for Acute Hearing Loss

2021 ◽  
Author(s):  
So-Young Jung ◽  
Zion Kang ◽  
Soonmin Kwon ◽  
Juhye Lee ◽  
Subin Kim ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Round Window ◽  
Round Window Membrane ◽  
In Vivo Test ◽  
Drug Concentrations ◽  
Intratympanic Injection ◽  
Acute Hearing Loss

Abstract Background: Dexamethasone sodium phosphate (Dex-SP) is the most commonly used drug for intratympanic injection in acute hearing loss, but its penetration efficiency into the inner ear is very low. To address this problem, we evaluated the possibility of dexamethasone nanosuspensions as intratympanic injection because the lipophilicity of drugs can affect their permeation of the round window membrane, an important pathway from the middle ear to the cochlea.Results: Three types of dexamethasone nanosuspensions were prepared; the dexamethasone nanocrystals in the three nanosuspensions were between approximately 250 and 350 nm in size. In order to compare the efficiency of Dex-SP and a dexamethasone nanosuspension in delivering dexamethasone to the inner ear, the concentrations of dexamethasone in perilymph and cochlear tissues were compared by liquid chromatography–mass spectrometry. The dexamethasone nanosuspensions showed significantly higher drug concentrations in perilymph and cochlear tissue than Dex-SP at 6 h; interestingly, animals treated with a nanosuspension showed a 26-fold higher dexamethasone concentration in the cochlear tissue than the Dex-SP group. In addition, the dexamethasone nanosuspension achieved better glucocorticoid receptor phosphorylation than Dex-SP both in vitro and in vivo, and in the ototoxic animal model, it showed a significantly better hearing protective effect than Dex-SP against ototoxic drugs. In safety evaluation, the nanosuspension showed no toxicity at concentrations up to 20 mg/mL in an in vivo test.Conclusions: A nanosuspension of dexamethasone was able to deliver dexamethasone to the cochlea very safely and efficiently and showed potential as a formula for intratympanic injection. In addition, it can be applied in studies on the delivery of various hydrophobic antioxidants to treat acute hearing loss.

Current indications for cochlear implantation in adults and children

2019 ◽  
Vol 73 (3) ◽  
pp. 1-5 ◽  
Author(s):  
Witold Szyfter ◽  
Michał Karlik ◽  
Alicja Sekula ◽  
Simon Harris ◽  
Wojciech Gawęcki
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Pure Tone Audiometry ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Unilateral Deafness ◽  
Bilateral Sensorineural Hearing Loss ◽  
Asymmetric Hearing Loss

Introduction: Surgical treatment of deafness by cochlear implants is used for more than 40 years, and during this period permanently, gradual and significant expansion of indications for this surgery has been observed. Material and methods: In our Department in the years 1994-2018 1480 cochlear implantations were performed, both in adults (647) and in children (883). In this study current indications and the rules for eligibility of patients based on 25 years of experience are presented. Results: Indications for cochlear implantation in adults are: 1) bilateral postlingual deafness, 2) bilateral sensorineural hearing loss - in pure tone audiometry > 70 dB HL (average 500-4000 Hz) and in speech audiometry in hearing aids understanding < 50% of words for the intensity of the stimulus 65 dB, in the absence of the benefits of hearing aids, 3) bilateral profound hearing loss for high frequency with good hearing for low frequency, in the absence of the benefits of hearing aids, 4) some cases of asymmetric hearing loss with intensive tinnitus in the deaf ear. An indication in children is bilateral sensorineural hearing loss > 80dB HL confirmed by hearing tests, after about 6 months of rehabilitation with the use of hearing aids. Discussion: Although cochlear implantation is used for more than 40 years, the indications for this treatment underlies constant modifications. They concern the age of eligible patients, implantation in patients with partially preserved hearing, as well as treatment for patients with difficult anatomical conditions. In many countries, bilateral implantations are commonly performed, and more and more centers recommend this treatment in the case of unilateral deafness or asymmetric hearing loss, especially with the accompanying tinnitus in the deaf ear.

scholarly journals Management of advanced otosclerosis: experience in a tertiary care centre

2018 ◽  
Vol 4 (2) ◽  
pp. 376
Author(s):  
Mohammed Iftekharul Alam ◽  
Kiran Natarajan ◽  
Takhellambam Biram Singh ◽  
Abha Kumari ◽  
Mohan Kameswaran
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Selection Criteria ◽  
Tertiary Care ◽  
Speech Discrimination ◽  
Tertiary Care Centre ◽  
Management Options ◽  
Profound Hearing Loss ◽  
Mixed Hearing Loss

<p class="abstract"><strong>Background:</strong> <span lang="EN-IN">Advanced otosclerosis affects approximately 10% of patients with otosclerosis. Ossification of the cochlea increases with the course of the disease and may cause sensori-neural or mixed hearing loss. Hearing aids, stapedotomy and cochlear implants are management options for hearing loss associated with advanced otosclerosis. </span></p><p class="abstract"><strong>Methods:</strong> <span lang="EN-IN">A retrospective study of 153 patients with advanced otosclerosis was done in a tertiary ENT centre. 110 patients with advanced otosclerosis underwent stapedotomy and 43 patients with advanced otosclerosis underwent cochlear implantation (CI) from 1997 till date. Exclusion criteria included patients with profound hearing loss from causes other than otosclerosis. The aim was to study the indications, selection criteria, as well as surgical issues of stapedotomy and cochlear implantation in patients with advanced otosclerosis.  </span></p><p class="abstract"><strong>Results:</strong> <span lang="EN-IN">Stapedotomy followed by use of hearing aid was found to give good outcomes in advanced otosclerosis. Patients with advanced otosclerosis who had poor pre-operative speech discrimination underwent CI. Complete insertion was possible in 39 patients and partial insertion was done in 4 patients. The outcomes of cochlear implantation were found to be satisfactory. Facial nerve stimulation was seen in 5 patients who underwent CI; however this was successfully managed by reprogramming or switching off the concerned electrodes. </span></p><p class="abstract"><strong>Conclusions:</strong> <span lang="EN-IN">In our experience, stapedotomy or CI in advanced otosclerosis has proven successful, with a low complication rate. The selection criteria for stapedotomy versus CI have to be stringent for optimal outcomes.</span></p>

scholarly journals Cochlear Implantation in Cases of Asymmetric Hearing Loss: Subjective Benefit, Word Recognition, and Spatial Hearing

2020 ◽  
Vol 24 ◽  
pp. 233121652094552
Author(s):  
Margaret T. Dillon ◽  
Emily Buss ◽  
Meredith A. Rooth ◽  
English R. King ◽  
Sarah A. McCarthy ◽  
...  
Keyword(s):  
Hearing Loss ◽  
Word Recognition ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Bone Conduction ◽  
Spatial Hearing ◽  
Published Data ◽  
Profound Hearing Loss ◽  
Hearing Sensitivity ◽  
Asymmetric Hearing Loss

A prospective clinical trial evaluated the effectiveness of cochlear implantation in adults with asymmetric hearing loss (AHL). Twenty subjects with mild-to-moderate hearing loss in the better ear and moderate-to-profound hearing loss in the poorer ear underwent cochlear implantation of the poorer hearing ear. Subjects were evaluated preoperatively and at 1, 3, 6, 9, and 12 months post-activation. Preoperative performance was evaluated unaided, with traditional hearing aids (HAs) or with a bone-conduction HA. Post-activation performance was evaluated with the cochlear implant (CI) alone or in combination with a contralateral HA (bimodal). Test measures included subjective benefit, word recognition, and spatial hearing (i.e., localization and masked sentence recognition). Significant subjective benefit was reported as early as the 1-month interval, indicating better performance with the CI compared with the preferred preoperative condition. Aided word recognition with the CI alone was significantly improved at the 1-month interval compared with preoperative performance with an HA and continued to improve through the 12-month interval. Subjects demonstrated early, significant improvements in the bimodal condition on the spatial hearing tasks compared with baseline preoperative performance tested unaided. The magnitude of the benefit was reduced for subjects with AHL when compared with published data on CI users with normal hearing in the contralateral ear; this finding may reflect significant differences in age at implantation and hearing sensitivity across cohorts.

Auditory and vestibular manifestations of Vogt–Koyanagi–Harada disease

2010 ◽  
Vol 125 (2) ◽  
pp. 138-141 ◽  
Author(s):  
S Al Dousary
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Cochlear Implantation ◽  
Organ Involvement ◽  
Disease Outcome ◽  
Profound Hearing Loss ◽  
Systemic Steroids ◽  
Chronic Disorder ◽  
High Incidence ◽  
Post Implantation

AbstractIntroduction and aims:Vogt–Koyanagi–Harada disease is a chronic disorder involving the eye and the central nervous, auditory, vestibular and integumentary systems. This study aimed to determine the auditory and vestibular manifestations of this disease.Methods:Twenty-four patients diagnosed with Vogt–Koyanagi–Harada disease were assessed for auditory and vestibular dysfunction.Results:Uveitis presents in all cases. Sensory hearing loss was present in 50 per cent of cases, tinnitus in 42 per cent, vertigo in 17 per cent and headache in 17 per cent. Nine patients received systemic steroids. Six patients who were treated early regained their hearing, but three patients whose treatment was delayed did not. One patient with bilateral profound hearing loss underwent cochlear implantation, and achieved excellent post-implantation hearing.Conclusion:There is a high incidence of cochlear and vestibular end-organ involvement in patients with Vogt–Koyanagi–Harada disease. The adequacy and timing of treatment has a significant effect on the disease outcome. Vogt–Koyanagi–Harada disease appears to affect the inner ear end-organ. Patients who develop bilateral profound sensory hearing loss are suitable candidates for cochlear implantation.

Congenital malformations of the inner ear

2021 ◽  
Vol 70 (3) ◽  
pp. 167-173
Author(s):  
Soňa Šikolová ◽  
Milan Urík ◽  
Jana Jančíková ◽  
Dagmar Hošnová ◽  
Rami Katra
Keyword(s):  
Hearing Loss ◽  
Inner Ear ◽  
Hearing Aids ◽  
Congenital Malformations ◽  
Profound Hearing Loss ◽  
Membranous Labyrinth ◽  
Bony Labyrinth ◽  
Tertiary Center ◽  
Hereditary Factors ◽  
And Function

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

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