Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post‐cochlear implantation outcomes

The stria vascularis in the C3H/ lpr autoimmune strain mouse was ultrastructurally examined in order to better understand the potential mechanisms by which systemic autoimmune disease affects the ear. The inner ears from C3H/ lpr mice before disease onset and C3H/HeJ controls showed no apparent pathology. However, the stria vascularis from older C3H/ lpr mice after systemic autoimmune disease onset showed considerable intercellular edema around the stria capillaries and thickening of the capillary basement membrane, compared to controls. These observations suggest that perivascular abnormalities, which are the hallmark of systemic autoimmune diseases, may underlie the stria dysfunction and hearing loss seen in autoimmune diseases in humans.

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Detailed Clinical Features of Deafness Caused by a Claudin-14 Variant

International Journal of Molecular Sciences ◽

10.3390/ijms20184579 ◽

2019 ◽

Vol 20 (18) ◽

pp. 4579 ◽

Cited By ~ 1

Author(s):

Kitano ◽

Kitajiri ◽

Nishio ◽

Usami

Keyword(s):

Hearing Loss ◽

Inner Ear ◽

Cochlear Implantation ◽

Vestibular Function ◽

Sound Field ◽

Speech Discrimination ◽

Epithelial Barrier Function ◽

Pathogenic Variants ◽

Novel Variant ◽

Claudin 14

Tight junctions are cellular junctions that play a major role in the epithelial barrier function. In the inner ear, claudins, occludin, tricellulin, and angulins form the bicellular or tricellular binding of membrane proteins. In these, one type of claudin gene, CLDN14, was reported to be responsible for human hereditary hearing loss, DFNB29. Until now, nine pathogenic variants have been reported, and most phenotypic features remain unclear. In the present study, genetic screening for 68 previously reported deafness causative genes was carried out to identify CLDN14 variants in a large series of Japanese hearing loss patients, and to clarify the prevalence and clinical characteristics of DFNB29 in the Japanese population. One patient had a homozygous novel variant (c.241C>T: p.Arg81Cys) (0.04%: 1/2549). The patient showed progressive bilateral hearing loss, with post-lingual onset. Pure-tone audiograms indicated a high-frequency hearing loss type, and the deterioration gradually spread to other frequencies. The patient showed normal vestibular function. Cochlear implantation improved the patient’s sound field threshold levels, but not speech discrimination scores. This report indicated that claudin-14 is essential for maintaining the inner ear environment and suggested the possible phenotypic expansion of DFNB29. This is the first report of a patient with a tight junction variant receiving a cochlear implantation.

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Exposure to silicates and systemic autoimmune-related outcomes in rodents: a systematic review

Particle and Fibre Toxicology ◽

10.1186/s12989-021-00439-6 ◽

2022 ◽

Vol 19 (1) ◽

Author(s):

Lisa M. F. Janssen ◽

Manosij Ghosh ◽

Frauke Lemaire ◽

K. Michael Pollard ◽

Peter H. M. Hoet

Keyword(s):

Systematic Review ◽

Autoimmune Disease ◽

Autoimmune Diseases ◽

Full Text ◽

Genetic Background ◽

Asbestos Exposure ◽

Systemic Autoimmune Disease ◽

Population Exposure ◽

Significant Heterogeneity ◽

Systemic Autoimmune Diseases

Abstract Background Autoimmunity can result from the interplay between genetic background and effects of environmental and/or occupational exposure to hazardous materials. Several compounds, including silica dust, have been linked with systemic autoimmunity and systemic autoimmune diseases, based on epidemiological evidence. For asbestos, a strong link with systemic autoimmune diseases does not yet exist, however, several studies have documented features of autoimmunity following asbestos exposure. Even so, human studies are limited in their ability to identify and examine isolated exposures, making it difficult to demonstrate causation or to assess pathogenic mechanisms. Therefore, this systematic review examines the existing animal evidence regarding autoimmunity and exposure to silicates (silica and asbestos). Methods PubMed and EMBASE were systematically searched for peer-reviewed studies examining systemic autoimmune disease-related outcomes after silicate exposure in rodents. Literature databases were searched up to September 2021 for studies written in English and where the full text was available. Search strings were established based on a PECO (Population, Exposure, Comparator, Outcome) format. After title, abstract, and full-text screening, thirty-four studies were identified for further analysis. Quality assessment through ToxR tool and qualitative analysis of the results was performed. Results Although there was significant heterogeneity in the included studies in terms of exposure protocol and genetic background of the rodent models used, it was noted that both genetic background and exposure to silicates [(crystalline) silica and asbestos] are highly relevant to the development of (sub-) clinical systemic autoimmune disease. Conclusion Parallels were observed between the findings from the animal (this review) and human (epidemiological) studies, arguing that experimental animal models are valuable tools for examining exacerbation or development of autoimmune disease after silicate exposure. However, genetic background and synergism between exposures should be considered in future studies.

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Predicting Sequential Cochlear Implantation Performance: A Systematic Review

Audiology and Neurotology ◽

10.1159/000488386 ◽

2017 ◽

Vol 22 (6) ◽

pp. 356-363 ◽

Cited By ~ 4

Author(s):

Yvette E. Smulders ◽

Thomas Hendriks ◽

Robert H. Eikelboom ◽

Inge Stegeman ◽

Peter L. Santa Maria ◽

...

Keyword(s):

Systematic Review ◽

Hearing Loss ◽

Speech Perception ◽

Cochlear Implantation ◽

Age At Onset ◽

Hearing Aid Use ◽

Long Duration ◽

Cochrane Database ◽

Expected Benefits ◽

Negative Factors

This systematic review of the literature reveals which preoperative factors affect sequential cochlear implantation outcomes in adults. The findings can help health care professionals provide evidence-based advice on the expected benefits from a second cochlear implant (CI). We searched PubMed, EMBASE, and the Cochrane database from November 1977 to August 26, 2017, using the terms “sequential cochlear implantation”; the most frequently cited predictors for unilateral cochlear implantation performance and other potential predictors for sequential implantation outcome; and “speech perception,” “localization” as well as synonyms of all of the above. Ten studies were included. The effects of age, duration of hearing loss, time between implantations, preoperative hearing, etiology of hearing loss, hearing aid use and duration of follow-up on sequential cochlear implantation performance were studied. The literature has shown that duration of deafness, age at onset of deafness, etiology of hearing loss, and preoperative speech perception score are (inversely) related to unilateral cochlear implantation outcome in adults. One would expect that these factors would also affect sequential bilateral implantation outcome. However, the best available evidence to date shows that advanced age, a long duration of deafness, or a long interval between implantations should not be considered negative factors when considering sequential bilateral cochlear implantation.

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Inner ear DNA receptors in MRL/lpr autoimmune mice: potential 30 and 70 kDa link between autoimmune disease and hearing loss

Hearing Research ◽

10.1016/s0378-5955(96)00191-8 ◽

1997 ◽

Vol 105 (1-2) ◽

pp. 57-64 ◽

Cited By ~ 22

Author(s):

Dennis R. Trune ◽

J. Beth Kempton ◽

Steven H. Hefeneider ◽

Robert M. Bennett

Keyword(s):

Hearing Loss ◽

Autoimmune Disease ◽

Inner Ear

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488 Anatomical and Audiological Considerations in Branchio-otic/Branchio-oto-renal Syndrome: A Systematic Review and Narrative Synthesis

British Journal of Surgery ◽

10.1093/bjs/znab134.560 ◽

2021 ◽

Vol 108 (Supplement_2) ◽

Author(s):

K Biggs ◽

G Crundwell ◽

C Metcalfe ◽

J Muzaffar ◽

P Monksfield ◽

...

Keyword(s):

Systematic Review ◽

Hearing Loss ◽

Hearing Aids ◽

Cochlear Implantation ◽

Middle Ear Surgery ◽

Narrative Synthesis ◽

Renal Anomalies ◽

Anatomical Variants ◽

Long Term Follow Up ◽

Ear Anomalies

Abstract Introduction Branchio-otic/ Branchio-oto-renal syndrome (BO/BOR) is a rare autosomal dominant condition characterised by hearing loss, branchiogenic and renal anomalies. Anatomical variants, audiological outcomes and optimal management are considered. Method Systematic review and narrative synthesis. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on year of publication. Results Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 503 patients from at least 104 families. In 308 patients hearing loss was categorised as sensorineural (29%), conductive (20%) and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity, ranging from mild to profound deafness. 43% patients presented with inner ear anomalies, 35% had middle and 36% had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however successful cochlear implantation was described in all five cases. Conclusions The anatomical and audiological profiles of patients with BO/BOR are variable. Cochlear implantation outcomes were good however the studies lacked long-term follow-up. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment.

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Sensorineural Hearing Loss and Systemic Autoimmune Disease: The Experience of a Systemic Immune-Mediated Diseases Unit

Cureus ◽

10.7759/cureus.14075 ◽

2021 ◽

Author(s):

Renata Ribeiro ◽

João F Serôdio ◽

Marta C Amaral ◽

Joana A Duarte ◽

Carolina Durão ◽

...

Keyword(s):

Hearing Loss ◽

Autoimmune Disease ◽

Sensorineural Hearing Loss ◽

Sensorineural Hearing ◽

Systemic Autoimmune Disease ◽

Immune Mediated

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Transduction Efficiency and Immunogenicity of Viral Vectors for Cochlear Gene Therapy: A Systematic Review of Preclinical Animal Studies

Frontiers in Cellular Neuroscience ◽

10.3389/fncel.2021.728610 ◽

2021 ◽

Vol 15 ◽

Author(s):

Dorien Verdoodt ◽

Noa Peeleman ◽

Guy Van Camp ◽

Vincent Van Rompaey ◽

Peter Ponsaerts

Keyword(s):

Systematic Review ◽

Gene Therapy ◽

Hearing Loss ◽

Sensorineural Hearing Loss ◽

Inner Ear ◽

Animal Studies ◽

Viral Vector ◽

Sensorineural Hearing ◽

Transduction Efficiency ◽

Preclinical Studies

Background: Hearing impairment is the most frequent sensory deficit, affecting 466 million people worldwide and has been listed by the World Health Organization (WHO) as one of the priority diseases for research into therapeutic interventions to address public health needs. Inner ear gene therapy is a promising approach to restore sensorineural hearing loss, for which several gene therapy applications have been studied and reported in preclinical animal studies.Objective: To perform a systematic review on preclinical studies reporting cochlear gene therapy, with a specific focus on transduction efficiency.Methods: An initial PubMed search was performed on April 1st 2021 using the PRISMA methodology. Preclinical in vivo studies reporting primary data regarding transduction efficiency of gene therapy targeting the inner ear were included in this report.Results: Thirty-six studies were included in this review. Transduction of various cell types in the inner ear can be achieved, according to the viral vector used. However, there is significant variability in the applied vector delivery systems, including promoter, viral vector titer, etc.Conclusion: Although gene therapy presents a promising approach to treat sensorineural hearing loss in preclinical studies, the heterogeneity of methodologies impedes the identification of the most promising tools for future use in inner ear therapies.

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A gyógyszeres kezelésre nem javuló hirtelen halláscsökkenés lépcsőzetes sebészi terápiája

Orvosi Hetilap ◽

10.1556/650.2021.32307 ◽

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.

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