Autoimmune hearing loss and a chance of its development in children : literature review and own observations
Autoimmune sensorineural hearing loss (AiSNHL) is an uncommon auditory disorder characterized by rapidly progressive bilateral hearing loss and a positive clinical response to treatment with corticosteroids and cytostatics. The prevalence of the disease in the adult population is less than 1% among all cases of subacute and permanent sensorineural hearing loss (precise data are unknown), it is even rarer in children. AiSNHL can be primary (isolated, organ-specific) or secondary (manifestation of another systemic autoimmune disease). The pathogenesis of AiSNHL is based on the proliferation of autoaggressive T cells and the pathological production of autoantibodies to the protein structures of the inner ear, which leads to damage to various parts of the cochlea (possibly also to the retrocochlear parts of the auditory system), less frequently to the vestibular labyrinth. Pathologically, the disease is most often represented by cochlear vasculitis with degeneration of the vascular stria, damage to hair cells and spiral ganglion cells, and endolymphatic hydrops. In 50% of cases, the result of autoimmune inflammation may be fibrosis and/or ossification of the cochlea. The most characteristic symptoms of AiSNHL at any age are episodes of sudden progression of hearing loss, fluctuations of hearing thresholds, and bilateral, often asymmetric impairments. The article presents contemporary ideas of the clinical and audiological manifestations of AiSNHL, the possibilities of diagnosing and treating the disease, and highlights the current approaches to (re)habilitation. Along with literature data, two own clinical cases of an extremely rare pediatric AiSNHL are given.
| Medienart: |
E-Artikel |
|---|
| Erscheinungsjahr: |
2023 |
|---|---|
| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:88 |
|---|---|
| Enthalten in: |
Vestnik otorinolaringologii - 88(2023), 1 vom: 01., Seite 77-84 |
| Sprache: |
Russisch |
|---|
| Weiterer Titel: |
Autoimmunnaya tugoukhost' i veroyatnost' ee formirovaniya v detskom vozraste: obzor literatury i sobstvennye nablyudeniya |
|---|
| Beteiligte Personen: |
Savenko, I V [VerfasserIn] |
|---|
| Links: |
|---|
| Themen: |
Autoimmune sensorineural hearing loss |
|---|
| Anmerkungen: |
Date Completed 07.03.2023 Date Revised 07.03.2023 published: Print Citation Status MEDLINE |
|---|
| doi: |
10.17116/otorino20228801177 |
|---|
| funding: |
|
|---|---|
| Förderinstitution / Projekttitel: |
|
| PPN (Katalog-ID): |
NLM353730513 |
|---|
| LEADER | 01000naa a22002652 4500 | ||
|---|---|---|---|
| 001 | NLM353730513 | ||
| 003 | DE-627 | ||
| 005 | 20231226060703.0 | ||
| 007 | cr uuu---uuuuu | ||
| 008 | 231226s2023 xx |||||o 00| ||rus c | ||
| 024 | 7 | |a 10.17116/otorino20228801177 |2 doi | |
| 028 | 5 | 2 | |a pubmed24n1179.xml |
| 035 | |a (DE-627)NLM353730513 | ||
| 035 | |a (NLM)36867148 | ||
| 040 | |a DE-627 |b ger |c DE-627 |e rakwb | ||
| 041 | |a rus | ||
| 100 | 1 | |a Savenko, I V |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Autoimmune hearing loss and a chance of its development in children |b literature review and own observations |
| 246 | 3 | 3 | |a Autoimmunnaya tugoukhost' i veroyatnost' ee formirovaniya v detskom vozraste: obzor literatury i sobstvennye nablyudeniya |
| 264 | 1 | |c 2023 | |
| 336 | |a Text |b txt |2 rdacontent | ||
| 337 | |a ƒaComputermedien |b c |2 rdamedia | ||
| 338 | |a ƒa Online-Ressource |b cr |2 rdacarrier | ||
| 500 | |a Date Completed 07.03.2023 | ||
| 500 | |a Date Revised 07.03.2023 | ||
| 500 | |a published: Print | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Autoimmune sensorineural hearing loss (AiSNHL) is an uncommon auditory disorder characterized by rapidly progressive bilateral hearing loss and a positive clinical response to treatment with corticosteroids and cytostatics. The prevalence of the disease in the adult population is less than 1% among all cases of subacute and permanent sensorineural hearing loss (precise data are unknown), it is even rarer in children. AiSNHL can be primary (isolated, organ-specific) or secondary (manifestation of another systemic autoimmune disease). The pathogenesis of AiSNHL is based on the proliferation of autoaggressive T cells and the pathological production of autoantibodies to the protein structures of the inner ear, which leads to damage to various parts of the cochlea (possibly also to the retrocochlear parts of the auditory system), less frequently to the vestibular labyrinth. Pathologically, the disease is most often represented by cochlear vasculitis with degeneration of the vascular stria, damage to hair cells and spiral ganglion cells, and endolymphatic hydrops. In 50% of cases, the result of autoimmune inflammation may be fibrosis and/or ossification of the cochlea. The most characteristic symptoms of AiSNHL at any age are episodes of sudden progression of hearing loss, fluctuations of hearing thresholds, and bilateral, often asymmetric impairments. The article presents contemporary ideas of the clinical and audiological manifestations of AiSNHL, the possibilities of diagnosing and treating the disease, and highlights the current approaches to (re)habilitation. Along with literature data, two own clinical cases of an extremely rare pediatric AiSNHL are given | ||
| 650 | 4 | |a English Abstract | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a autoimmune sensorineural hearing loss | |
| 650 | 4 | |a cochlear implantation | |
| 650 | 4 | |a hearing aids | |
| 650 | 4 | |a systemic autoimmune diseases | |
| 700 | 1 | |a Boboshko, M Yu |e verfasserin |4 aut | |
| 700 | 1 | |a Garbaruk, E S |e verfasserin |4 aut | |
| 700 | 1 | |a Nazarov, V D |e verfasserin |4 aut | |
| 700 | 1 | |a Tkachenko, O Yu |e verfasserin |4 aut | |
| 700 | 1 | |a Lapin, S V |e verfasserin |4 aut | |
| 773 | 0 | 8 | |i Enthalten in |t Vestnik otorinolaringologii |d 1945 |g 88(2023), 1 vom: 01., Seite 77-84 |w (DE-627)NLM000085405 |x 0042-4668 |7 nnns |
| 773 | 1 | 8 | |g volume:88 |g year:2023 |g number:1 |g day:01 |g pages:77-84 |
| 856 | 4 | 0 | |u http://dx.doi.org/10.17116/otorino20228801177 |3 Volltext |
| 912 | |a GBV_USEFLAG_A | ||
| 912 | |a GBV_NLM | ||
| 951 | |a AR | ||
| 952 | |d 88 |j 2023 |e 1 |b 01 |h 77-84 | ||