Cochlear implant in immune mediated inner ear diseases: Impedance variations and clinical outcomes

AbstractAutoimmune retinopathy (AIR) is a rare immune-mediated inflammation of the retina. The autoantibodies against retinal proteins and glycolytic enzymes were reported to be involved in the pathogenesis. This retrospective cohort study assessed the antiretinal autoantibody profiles and their association with clinical outcomes of AIR patients in Thailand. We included 44 patients, 75% were females, with the overall median age of onset of 48 (17–74, IQR 40–55.5) years. Common clinical presentations were nyctalopia (65.9%), blurred vision (52.3%), constricted visual field (43.2%), and nonrecordable electroretinography (65.9%). Underlying malignancy and autoimmune diseases were found in 2 and 12 female patients, respectively. We found 41 autoantibodies, with anti-α-enolase (65.9%) showing the highest prevalence, followed by anti-CAII (43.2%), anti-aldolase (40.9%), and anti-GAPDH (36.4%). Anti-aldolase was associated with male gender (P = 0.012, OR 7.11, 95% CI 1.54–32.91). Anti-CAII showed significant association with age of onset (P = 0.025, 95% CI − 17.28 to − 1.24), while anti-α-enolase (P = 0.002, OR 4.37, 95% CI 1.83–10.37) and anti-GAPDH (P = 0.001, OR 1.87, 95% CI 1.32–2.64) were significantly associated with nonrecordable electroretinography. Association between the antibody profiles and clinical outcomes may be used to direct and adjust the treatment plans and provide insights in the pathogenesis of AIR.

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Ultrasound Microbubbles Enhance the Efficacy of Insulin-Like Growth Factor-1 Therapy for the Treatment of Noise-Induced Hearing Loss

Molecules ◽

10.3390/molecules26123626 ◽

2021 ◽

Vol 26 (12) ◽

pp. 3626

Author(s):

Yi-Chun Lin ◽

Yuan-Yung Lin ◽

Hsin-Chien Chen ◽

Chao-Yin Kuo ◽

Ai-Ho Liao ◽

...

Keyword(s):

Hearing Loss ◽

Growth Factor ◽

Inner Ear ◽

Outer Hair Cells ◽

Round Window Membrane ◽

Insulin Like Growth Factor ◽

Noise Induced Hearing Loss ◽

Ear Diseases ◽

Brainstem Response ◽

Ultrasound Microbubbles

The application of insulin-like growth factor 1 (IGF-1) to the round window membrane (RWM) is an emerging treatment for inner ear diseases. RWM permeability is the key factor for efficient IGF-1 delivery. Ultrasound microbubbles (USMBs) can increase drug permeation through the RWM. In the present study, the enhancing effect of USMBs on the efficacy of IGF-1 application and the treatment effect of USMB-mediated IGF-1 delivery for noise-induced hearing loss (NIHL) were investigated. Forty-seven guinea pigs were assigned to three groups: the USM group, which received local application of recombinant human IGF-1 (rhIGF-1, 10 µg/µL) following application of USMBs to the RWM; the RWS group, which received IGF-1 application alone; and the saline-treated group. The perilymphatic concentration of rhIGF-1 in the USM group was 1.95- and 1.67- fold of that in the RWS group, 2 and 24 h after treatment, respectively. After 5 h of 118 dB SPL noise exposure, the USM group had the lowest threshold shift in auditory brainstem response, least loss of cochlear outer hair cells, and least reduction in the number of synaptic ribbons on postexposure day 28 among the three groups. The combination of USMB and IGF-1 led to a better therapeutic response to NIHL. Two hours after treatment, the USM group had significantly higher levels of Akt1 and Mapk3 gene expression than the other two groups. The most intense immunostaining for phosphor-AKT and phospho-ERK1/2 was detected in the cochlea in the USM group. These results suggested that USMB can be applied to enhance the efficacy of IGF-1 therapy in the treatment of inner ear diseases.

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Immune-mediated inner ear disease

Current Opinion in Rheumatology ◽

10.1097/00002281-200001000-00006 ◽

2000 ◽

Vol 12 (1) ◽

pp. 32-40 ◽

Cited By ~ 73

Author(s):

John H. Stone ◽

Howard W. Francis

Keyword(s):

Inner Ear ◽

Immune Mediated ◽

Inner Ear Disease

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Cochlear Implantation Outcomes in Patients With Autoimmune and Immune-Mediated Inner Ear Disease

Otology & Neurotology ◽

10.1097/mao.0b013e3181f0c699 ◽

2010 ◽

Vol 31 (8) ◽

pp. 1337-1342 ◽

Cited By ~ 22

Author(s):

Saba Aftab ◽

Maroun T. Semaan ◽

Gail S. Murray ◽

Cliff A. Megerian

Keyword(s):

Inner Ear ◽

Cochlear Implantation ◽

Immune Mediated ◽

Inner Ear Disease

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The Influence of a Cochlear Implant Electrode on the Mechanical Function of the Inner Ear

Otology & Neurotology ◽

10.1097/mao.0b013e3181ca372b ◽

2010 ◽

Vol 31 (3) ◽

pp. 512-518 ◽

Cited By ~ 20

Author(s):

Alexander M. Huber ◽

Sim Jae Hoon ◽

Bonabi Sharouz ◽

Bodmer Daniel ◽

Eiber Albrecht

Keyword(s):

Cochlear Implant ◽

Inner Ear ◽

Mechanical Function

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A new cochlear implant electrode with a “cork”-type stopper for inner ear malformations

Auris Nasus Larynx ◽

10.1016/j.anl.2013.12.011 ◽

2014 ◽

Vol 41 (4) ◽

pp. 331-336 ◽

Cited By ~ 18

Author(s):

Levent Sennaroğlu ◽

Gamze Atay ◽

Münir Demir Bajin

Keyword(s):

Cochlear Implant ◽

Inner Ear ◽

Inner Ear Malformations ◽

Ear Malformations

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In Situ 3D-Imaging of the Inner Ear Synapses with a Cochlear Implant

Life ◽

10.3390/life11040301 ◽

2021 ◽

Vol 11 (4) ◽

pp. 301

Author(s):

Kathrin Malfeld ◽

Nina Armbrecht ◽

Holger A. Volk ◽

Thomas Lenarz ◽

Verena Scheper

Keyword(s):

Cochlear Implant ◽

Inner Ear ◽

Laser Scanning ◽

3D Imaging ◽

Laser Scanning Microscopy ◽

Confocal Laser ◽

Sensory Cells ◽

Residual Hearing ◽

Preparation Methods

In recent years sensorineural hearing loss was found to affect not exclusively, nor at first, the sensory cells of the inner ear. The sensory cells’ synapses and subsequent neurites are initially damaged. Auditory synaptopathies also play an important role in cochlear implant (CI) care, as they can lead to a loss of physiological hearing in patients with residual hearing. These auditory synaptopathies and in general the cascades of hearing pathologies have been in the focus of research in recent years with the aim to develop more targeted and individually tailored therapeutics. In the current study, a method to examine implanted inner ears of guinea pigs was developed to examine the synapse level. For this purpose, the cochlea is made transparent and scanned with the implant in situ using confocal laser scanning microscopy. Three different preparation methods were compared to enable both an overview image of the cochlea for assessing the CI position and images of the synapses on the same specimen. The best results were achieved by dissection of the bony capsule of the cochlea.

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Role of Computed Tomography and Magnetic Resonance Imaging in detecting the Prevalence of inner ear anomalies among cochlear implant candidates

QJM ◽

10.1093/qjmed/hcab106.007 ◽

2021 ◽

Vol 114 (Supplement_1) ◽

Author(s):

Alaa Nasser Hussain Zaher ◽

Tougan Taha Abd El Aziz ◽

Ahmed Samy Abdelrahman

Keyword(s):

Magnetic Resonance Imaging ◽

Computed Tomography ◽

Hearing Loss ◽

Cochlear Implant ◽

Inner Ear ◽

Resonance Imaging ◽

High Resolution Computed Tomography ◽

Vestibular Aqueduct ◽

Incomplete Partition ◽

Ear Anomalies

Abstract Background Hearing loss management using cochlear implants in patients with inner ear anomalies has long been discussed in the otology community. Magnetic resonances imaging (B,/IRI) and Computed tomography (CT) play important roles in the preoperative assessment of inner ear abnormalities such as cochlear nerve deficiency and variant anatomy as these abnormalities may not only affect the decision of the implantation procedure or the patient's prognosis regarding auditory improvement, but also the risk of complications. Objective To examine the prevalence of inner ear anomalies among cochlear implant recipients in patients with congenital sensorineural hearing loss among the pediatric age group in the Demerdash hospital, Ain Shams university using High resolution computed tomography (HRCT) and MRI imaging. Methods A retrospective descriptive study over the course of 9 months that included all patients that are candidates for cochlear implant referred to the Radiology department, Ain Shams University Hospitals for a preoperative imaging in the form of CT and VIRI scans. Results CT and MRI scans of 33 patients who had congenital hearing loss and were candidates for cochlear implantation with total 66 ears were reviewed. Inner ear anomalies were identified in 8 patients representing a prevalence (24.2%) with 14 ear diseased. Anomalies were seen bilaterally in 6 patients and unilaterally in 2 patients. Among the 14 diseased ear, 9 ears (64.3%) were seen with incomplete partition Il, 7 ears (50%) were seen with enlarged vestibular aqueduct, 4 ears (28.6%) were seen with cochlear hypoplasia, 3 ears (21.4%) were seen with semicircular canal aplasia, 2 ears (14.3%) were seen with incomplete partition type I, 2 ears (14.3%) were seen with cochlear nerve aplasia, 2 ears with cochlear aplasia (14.3%), I ear (7.1%) was seen with common cavity ear (7.1%) with complete labyrinthine aplasia. Conclusion Prevalence of inner ear anomalies among cochlear implant candidates was 24.2%. This result is consistent with results worldwide and the most common anomalies were Incomplete partition Il and large vestibular aqueduct. Abbreviations Computed tomography (CT), Magnetic resonance imaging (MRI), High resolution computed tomography (HRCT), Internal auditory canal (IAC), Cerebellopontine angle (CPA).

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