Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis (EBV-HLH) is a life-threatening catastrophic and rarely seen complication of EBV infection especially in adults. While typical presentation of EBV infection is easily diagnosed as mononucleosis syndrome in teenagers and adults, some atypical clinical presentations may be challenged. We did not encounter any patient presenting with sudden sensorineural hearing loss associated with EBV infection in our English medical literature research (1966-2016). In this study, we report an adult patient who was complicated with EBV-HLH under high dose steroid therapy after diagnosis as sensorineural hearing loss. Our aim is to emphasise the atypical presentation of EBV infection and to discuss steroid therapy complication in sensorineural hearing loss that had been simply defined as idiopathic.
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Diabetol Int
January 2025
Department of Endocrinology and Diabetes, School of Medicine, Saitama Medical University, 38 Morohongo, Moroyamamachi, Iruma-Gun, Saitama, 350-0495 Japan.
Slowly progressive type 1 diabetes mellitus (SPIDDM), solely positive for zinc transporter 8 autoantibody (ZnT8A) is rare, and the factors involved in the single positivity remain largely unknown. Thus, this case report aimed to infer the factors based on a literature review. A 40-year-old female was hospitalized for hyperglycemia.
View Article and Find Full Text PDFMol Genet Genomic Med
February 2025
Medical Genetics, Department of Precision Medicine, University of Campania 'Luigi Vanvitelli', Naples, Italy.
Background: Sensorineural hearing loss (SNHL) is a frequent manifestation of syndromic inherited retinal diseases (IRDs), exemplified by the very rare form of autosomal-dominant Leber congenital amaurosis with early onset deafness (LCAEOD; OMIM #617879). LCAEOD was first described in 2017 in four families segregating heterozygous missense mutations in TUBB4B, a gene encoding a β-tubulin isotype. To date, only eight more families with similar TUBB4B-associated sensorineural disease (SND) have been reported.
View Article and Find Full Text PDFEar Nose Throat J
January 2025
Otorhinolaryngology Department, Affiliated Hospital of Hangzhou Normal University, Hangzhou, Zhejiang Province, China.
This retrospective study aimed to compare the efficacy of portable ear endoscopic tympanic injection versus conventional ear endoscopic tympanic injection in the treatment of idiopathic sudden sensorineural hearing loss (ISSNHL) and discusses the advantages and disadvantages of each method. Clinical data of 128 patients with ISSNHL who did not respond to conventional treatment were collected from January 2019 to June 2023. Patients were divided into 2 groups: Group 1 (n = 60) received portable ear endoscopic tympanic injection, while Group 2 (n = 68) received conventional ear endoscopic tympanic injection.
View Article and Find Full Text PDFIndian J Nephrol
June 2024
Mansoura Nephrology and Dialysis Unit, Department of Internal Medicine, Mansoura, Egypt.
Background: It has been claimed that tacrolimus may have harmful effects on the auditory system, where it has been linked to ototoxicity and sensorineural hearing loss (SNHL). We evaluated silent SNHL in kidney transplant recipients (KTRs) receiving tacrolimus and the different factors affecting it compared to healthy controls.
Materials And Methods: In this case control study, hearing functions were studied in 42 KTRs receiving tacrolimus as maintenance immunosuppressive therapy for more than 3 months in comparison to 27 age- and gender-matched healthy subjects using tympanometry, pure-tone audiometry (PTA), extended high frequency audiometry (EHFA), and transient evoked oto-acoustic emissions (TEOAEs).
Cell Commun Signal
January 2025
Department of Otolaryngology-Head and Neck Surgery, Shandong Provincial ENT Hospital, Shandong University, Jinan, 250022, China.
Degeneration of cochlear spiral ganglion neurons (SGNs) leads to irreversible sensorineural hearing loss (SNHL), as SGNs lack regenerative capacity. Although cochlear glial cells (GCs) have some neuronal differentiation potential, their specific identities remain unclear. This study identifies a distinct subpopulation, Frizzled10 positive (FZD10+) cells, as an important type of GC responsible for neuronal differentiation in mouse cochlea.
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