Bilateral Fluctuating Sensorineural Hearing Loss as a Paraneoplastic Manifestation for Lymphoma

Autoimmune inner ear disease (AIED) is an uncommon cause of fluctuating sensorineural hearing loss (SNHL), which is diagnosed by exclusion and required a high index of suspicion. Secondary AIED, which accompanies other autoimmune diseases, occurs in up to 30% of AIED cases. Secondary AIED is a rare...

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Published inThe Laryngoscope Vol. 135; no. 4; p. 1496
Main Authors Young, Ting-Chia, Wang, Wen-Hsiu, Chu, Chia-Huei, Lin, Hung-Ching, Chen, Pey-Yu
Format Journal Article
LanguageEnglish
Published United States 01.04.2025
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Abstract Autoimmune inner ear disease (AIED) is an uncommon cause of fluctuating sensorineural hearing loss (SNHL), which is diagnosed by exclusion and required a high index of suspicion. Secondary AIED, which accompanies other autoimmune diseases, occurs in up to 30% of AIED cases. Secondary AIED is a rare manifestation of paraneoplastic syndrome, and the causal malignancy may be occult initially. Detecting and treating associated diseases, along with the use of immunosuppressants, are the mainstay for the management of AIED. Herein, we presented a woman with bilateral fluctuating and progressive SNHL, partially responding to steroid or immunosuppressant treatment. Her hearing eventually stabilized after completing the treatment for diffuse large B cell lymphoma, which was diagnosed 6 months after the onset of hearing loss. This case serves as a reminder to physicians to be aware of AIED in cases of fluctuating SNHL and the possibility of associated malignancy alongside autoimmune disturbance. Laryngoscope, 135:1496-1498, 2025.
AbstractList Autoimmune inner ear disease (AIED) is an uncommon cause of fluctuating sensorineural hearing loss (SNHL), which is diagnosed by exclusion and required a high index of suspicion. Secondary AIED, which accompanies other autoimmune diseases, occurs in up to 30% of AIED cases. Secondary AIED is a rare manifestation of paraneoplastic syndrome, and the causal malignancy may be occult initially. Detecting and treating associated diseases, along with the use of immunosuppressants, are the mainstay for the management of AIED. Herein, we presented a woman with bilateral fluctuating and progressive SNHL, partially responding to steroid or immunosuppressant treatment. Her hearing eventually stabilized after completing the treatment for diffuse large B cell lymphoma, which was diagnosed 6 months after the onset of hearing loss. This case serves as a reminder to physicians to be aware of AIED in cases of fluctuating SNHL and the possibility of associated malignancy alongside autoimmune disturbance. Laryngoscope, 135:1496-1498, 2025.
Author Lin, Hung-Ching
Chen, Pey-Yu
Wang, Wen-Hsiu
Young, Ting-Chia
Chu, Chia-Huei
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  surname: Chen
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  organization: Department of Otolaryngology, National Taiwan University Hospital, Taipei, Taiwan
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Keywords paraneoplastic syndromes
acute sensorineural hearing loss
autoimmune inner ear disease
lymphoma
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Snippet Autoimmune inner ear disease (AIED) is an uncommon cause of fluctuating sensorineural hearing loss (SNHL), which is diagnosed by exclusion and required a high...
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StartPage 1496
SubjectTerms Adult
Autoimmune Diseases - complications
Autoimmune Diseases - diagnosis
Autoimmune Diseases - drug therapy
Autoimmune Diseases - etiology
Female
Hearing Loss, Bilateral - diagnosis
Hearing Loss, Bilateral - etiology
Hearing Loss, Sensorineural - diagnosis
Hearing Loss, Sensorineural - etiology
Humans
Lymphoma, Large B-Cell, Diffuse - complications
Lymphoma, Large B-Cell, Diffuse - diagnosis
Lymphoma, Large B-Cell, Diffuse - drug therapy
Paraneoplastic Syndromes - diagnosis
Paraneoplastic Syndromes - etiology
Title Bilateral Fluctuating Sensorineural Hearing Loss as a Paraneoplastic Manifestation for Lymphoma
URI https://www.ncbi.nlm.nih.gov/pubmed/39440532
Volume 135
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