{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T13:34:50Z","timestamp":1770471290667,"version":"3.49.0"},"reference-count":87,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T00:00:00Z","timestamp":1760227200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Hyper-IgE syndrome (HIES) is a rare genetic immunodeficiency characterized by elevated serum IgE levels and associated immune dysregulation, manifesting in recurrent infections, eczema, and skeletal abnormalities. Emerging evidence suggests a link between HIES and audiovestibular dysfunction, potentially mediated by IgE-driven inflammation in the inner ear, which is not immunologically privileged. However, the nature of this association remains poorly understood. This systematic review synthesizes current evidence on the characteristics, pathophysiology, diagnostic approaches, and management of audiovestibular dysfunction in HIES patients. Literature searches across PubMed, Embase, ClinicalKey, Web of Science, and ScienceDirect (up to 6 August 2025) were conducted in accordance with PRISMA guidelines. Key findings indicate that HIES-related audiovestibular issues, including sensorineural hearing loss and vestibular impairment, may arise from IgE-mediated endolymphatic sac inflammation, leading to hydrops and hair cell damage. Diagnostic tools such as audiometry, electrocochleography, and allergen challenge tests show promise, with elevated IgE correlating with abnormal otoacoustic emissions and prolonged auditory brainstem response latencies. Treatment focuses on immunomodulation (e.g., corticosteroids, dupilumab) to mitigate IgE effects, though evidence is limited to case reports. A proposed schematic diagram illustrates pathophysiology, emphasizing IgE\u2019s role in inner ear toxicity. Timely recognition and intervention may prevent progression to permanent hearing loss or vestibular disability, improving quality of life. Future research should explore genetic\u2013immunologic mechanisms and prospective trials for targeted therapies. Trial registration: PROSPERO CRD420251120600.<\/jats:p>","DOI":"10.3390\/ijms26209932","type":"journal-article","created":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T16:13:03Z","timestamp":1760717583000},"page":"9932","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Audiovestibular Dysfunction in Hyper-IgE Syndrome: A Systematic Review of Characteristics, Pathophysiology, Diagnosis, and Management"],"prefix":"10.3390","volume":"26","author":[{"given":"Jiann-Jy","family":"Chen","sequence":"first","affiliation":[{"name":"Prospect Clinic for Otorhinolaryngology & Neurology, Kaohsiung 81166, Taiwan"},{"name":"Department of Otorhinolaryngology, E-Da Cancer Hospital, I-Shou University, Kaohsiung 82445, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8650-4060","authenticated-orcid":false,"given":"Chih-Wei","family":"Hsu","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung 833401, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brendon","family":"Stubbs","sequence":"additional","affiliation":[{"name":"Department of Psychological Medicine, Institute of Psychiatry, Psychology and Neuroscience, King\u2019s College London, London WC2R 2LS, UK"},{"name":"Department of Sport, University of Vienna, 1010 Vienna, Austria"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8462-1311","authenticated-orcid":false,"given":"Tien-Yu","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, Tri-Service General Hospital, Taipei 114202, Taiwan"},{"name":"Department of Psychiatry, College of Medicine, National Defense Medical University, Taipei 114202, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1138-5586","authenticated-orcid":false,"given":"Chih-Sung","family":"Liang","sequence":"additional","affiliation":[{"name":"Department of Psychiatry, Beitou Branch, Tri-Service General Hospital, School of Medicine, National Defense Medical University, Taipei 11243, Taiwan"},{"name":"Department of Psychiatry, National Defense Medical University, Taipei 114202, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yen-Wen","family":"Chen","sequence":"additional","affiliation":[{"name":"Prospect Clinic for Otorhinolaryngology & Neurology, Kaohsiung 81166, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bing-Yan","family":"Zeng","sequence":"additional","affiliation":[{"name":"Institute of Biomedical Sciences, National Sun Yat-sen University, Kaohsiung 80424, Taiwan"},{"name":"Department of Internal Medicine, E-Da Dachang Hospital, I-Shou University, Kaohsiung 807066, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5761-7800","authenticated-orcid":false,"given":"Ping-Tao","family":"Tseng","sequence":"additional","affiliation":[{"name":"Prospect Clinic for Otorhinolaryngology & Neurology, Kaohsiung 81166, Taiwan"},{"name":"Institute of Biomedical Sciences, National Sun Yat-sen University, Kaohsiung 80424, Taiwan"},{"name":"Department of Psychology, College of Medical and Health Science, Asia University, Taichung 406040, Taiwan"},{"name":"Institute of Precision Medicine, National Sun Yat-sen University, Kaohsiung 80424, Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,10,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1016\/j.iac.2008.01.005","article-title":"The hyper-IgE syndromes","volume":"28","author":"Freeman","year":"2008","journal-title":"Immunol. Allergy Clin. N. Am."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1016\/S0140-6736(66)90119-X","article-title":"Job\u2032s Syndrome. Recurrent, \u201ccold\u201d, staphylococcal abscesses","volume":"1","author":"Davis","year":"1966","journal-title":"Lancet"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1542\/peds.49.1.59","article-title":"Extreme hyperimmunoglobulinemia E and undue susceptibility to infection","volume":"49","author":"Buckley","year":"1972","journal-title":"Pediatrics"},{"key":"ref_4","unstructured":"Hafsi, W., and Yarrarapu, S.N.S. (2025). Job Syndrome. StatPearls, StatPearls Publishing."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"e70497","DOI":"10.1002\/hsr2.70497","article-title":"Hyper IgE Syndromes: Understanding, Management, and Future Perspectives: A Narrative Review","volume":"8","author":"Salehi","year":"2025","journal-title":"Health Sci. Rep."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1288\/00005537-199103000-00001","article-title":"Meniere\u2032s disease: An immune complex-mediated illness?","volume":"101","author":"Derebery","year":"1991","journal-title":"Laryngoscope"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1795","DOI":"10.1007\/s00405-012-2189-y","article-title":"The role of allergy in the etiopathogenesis of idiopathic sudden sensorineural hearing loss","volume":"270","author":"Keles","year":"2013","journal-title":"Eur. Arch. Otorhinolaryngol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"827","DOI":"10.4049\/jimmunol.2100745","article-title":"Bidirectional Transport of IgE by CD23 in the Inner Ear of Patients with Meniere\u2032s Disease","volume":"208","author":"Zhang","year":"2022","journal-title":"J. Immunol."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Tseng, P.T., Chen, T.Y., Lui, C.C., Chen, Y.W., and Chen, J.J. (2022). Tinnitus Associated with Mild Osteomyelitis of the Temporal Bone Reversed after Conservative Antibiotic Treatment: A Case Series. Medicina, 58.","DOI":"10.3390\/medicina58020318"},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Chen, J.J., Hsu, C.W., Chen, Y.W., Chen, T.Y., Zeng, B.Y., and Tseng, P.T. (2024). Audiovestibular Dysfunction Related to Anti-Phospholipid Syndrome: A Systematic Review. Diagnostics, 14.","DOI":"10.3390\/diagnostics14222522"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Chen, J.J., Hsu, C.W., Chen, Y.W., Chen, T.Y., Zeng, B.S., and Tseng, P.T. (2024). Audiovestibular Dysfunction in Systemic Lupus Erythematosus Patients: A Systematic Review. Diagnostics, 14.","DOI":"10.3390\/diagnostics14151670"},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Chen, J.J., Hsu, C.W., Chen, Y.W., Chen, T.Y., Zeng, B.S., and Tseng, P.T. (2024). Audiological Features in Patients with Rheumatoid Arthritis: A Systematic Review. Int. J. Mol. Sci., 25.","DOI":"10.3390\/ijms252413290"},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Chen, J.J., Hsu, C.W., Chen, T.Y., Liang, C.S., Chen, Y.W., Zeng, B.Y., and Tseng, P.T. (2025). Audiovestibular Dysfunction in Patients with Hashimoto\u2032s Disease: A Systematic Review. Int. J. Mol. Sci., 26.","DOI":"10.3390\/ijms26104703"},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Maihoub, S., Mavrogeni, P., Molnar, V., and Molnar, A. (2025). Tinnitus and Its Comorbidities: A Comprehensive Analysis of Their Relationships. J. Clin. Med., 14.","DOI":"10.3390\/jcm14041285"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1258\/0022215054273133","article-title":"Co-morbidity of migraine and Meniere\u2019s disease\u2014Is allergy the link?","volume":"119","author":"Sen","year":"2005","journal-title":"J. Laryngol. Otol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.alit.2021.07.007","article-title":"Hyper-IgE syndrome, 2021 update","volume":"70","author":"Minegishi","year":"2021","journal-title":"Allergol. Int."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/j.amjoto.2006.12.003","article-title":"Expression of histamine receptors (H(1), H(2), and H(3)) in the rabbit endolymphatic sac: An immunohistochemical study","volume":"29","author":"Dagli","year":"2008","journal-title":"Am. J. Otolaryngol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1159\/000276124","article-title":"Human endolymphatic sac: Evidence for a role in inner ear immune defence","volume":"52","author":"Altermatt","year":"1990","journal-title":"ORL J. Otorhinolaryngol. Relat. Spec."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"283","DOI":"10.1016\/S0194-5998(99)70186-5","article-title":"Electrocochleographic changes after intranasal allergen challenge: A possible diagnostic tool in patients with Meniere\u2032s disease","volume":"121","author":"Gibbs","year":"1999","journal-title":"Otolaryngol.-Head Neck Surg."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1177\/000348940411300605","article-title":"Immune-mediated disorders associated with idiopathic sudden sensorineural hearing loss","volume":"113","author":"Toubi","year":"2004","journal-title":"Ann. Otol. Rhinol. Laryngol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1258\/0022215042244822","article-title":"Meniere\u2032s disease and allergy: Allergens and cytokines","volume":"118","author":"Keles","year":"2004","journal-title":"J. Laryngol. Otol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"102856","DOI":"10.1016\/j.amjoto.2020.102856","article-title":"High level of IgE in acute low-tone sensorineural hearing loss: A predictor for recurrence and Meniere Disease transformation","volume":"42","author":"Ma","year":"2021","journal-title":"Am. J. Otolaryngol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1016\/S0196-0709(99)90064-9","article-title":"Immunologic aspects of Meniere\u2032s disease","volume":"20","author":"Ruckenstein","year":"1999","journal-title":"Am. J. Otolaryngol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"125","DOI":"10.1007\/s12070-020-01890-1","article-title":"Hearing Assessment in Patients of Allergic Rhinitis: A Study on 200 Subjects","volume":"74","author":"Sahni","year":"2022","journal-title":"Indian. J. Otolaryngol.-Head Neck Surg."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1067\/mhn.2000.105715","article-title":"Prevalence of allergy in Meniere\u2032s disease","volume":"123","author":"Derebery","year":"2000","journal-title":"Otolaryngol.-Head Neck Surg."},{"key":"ref_26","first-page":"903","article-title":"The inner ear in patients with nasal allergy","volume":"100","author":"Lasisi","year":"2008","journal-title":"J. Natl. Med. Assoc."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"174","DOI":"10.1016\/S0194-5998(00)70235-X","article-title":"Allergic management of Meniere\u2032s disease: An outcome study","volume":"122","author":"Derebery","year":"2000","journal-title":"Otolaryngol.-Head Neck Surg."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"n71","DOI":"10.1136\/bmj.n71","article-title":"The PRISMA 2020 statement: An updated guideline for reporting systematic reviews","volume":"372","author":"Page","year":"2021","journal-title":"BMJ"},{"key":"ref_29","unstructured":"Wells, G.A., Shea, B., O\u2032Connell, D., Peterson, J., Welch, V., Losos, M., and Tugwell, P. (2024, June 12). The Newcastle-Ottawa Scale (NOS) for Assessing the Quality of Nonrandomised Studies in Meta-Analyses. Available online: https:\/\/www.ohri.ca\/programs\/clinical_epidemiology\/oxford.asp."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1086\/302547","article-title":"Genetic linkage of hyper-IgE syndrome to chromosome 4","volume":"65","author":"Grimbacher","year":"1999","journal-title":"Am. J. Hum. Genet."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1097\/MS9.0000000000001670","article-title":"Hyper-IgE syndrome: A case report","volume":"86","author":"Dave","year":"2024","journal-title":"Ann. Med. Surg. (Lond.)"},{"key":"ref_32","first-page":"53","article-title":"Hyperimmunoglobulin E syndrome: Genetics, immunopathogenesis, clinical findings, and treatment modalities","volume":"22","author":"Hashemi","year":"2017","journal-title":"J. Res. Med. Sci."},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"AlYafie, R., Velayutham, D., van Panhuys, N., and Jithesh, P.V. (2025). The genetics of hyper IgE syndromes. Front. Immunol., 16.","DOI":"10.3389\/fimmu.2025.1516068"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/s11882-025-01196-8","article-title":"Hyper IgE Syndrome: Bridging the Gap Between Immunodeficiency, Atopy, and Allergic Diseases","volume":"25","author":"Sutanto","year":"2025","journal-title":"Curr. Allergy Asthma Rep."},{"key":"ref_35","doi-asserted-by":"crossref","unstructured":"Yaakoubi, R., Mekki, N., Ben-Mustapha, I., Ben-Khemis, L., Bouaziz, A., Ben Fraj, I., Ammar, J., Hamzaoui, A., Turki, H., and Boussofara, L. (2022). Diagnostic challenge in a series of eleven patients with hyper IgE syndromes. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.1057679"},{"key":"ref_36","doi-asserted-by":"crossref","unstructured":"Lachover-Roth, I., Lagovsky, I., Shtorch-Asor, A., Confino-Cohen, R., Reinstein, E., and Garty, B.Z. (2022). Hyper IgE Syndrome in an Isolated Population in Israel. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.829239"},{"key":"ref_37","first-page":"8","article-title":"A Review on Hyper-IgE Syndromes, Clinical Manifestations, Diagnosis and Therapeutic Approaches","volume":"2","author":"Tavakol","year":"2019","journal-title":"Immunol. Genet. J."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"omaa106","DOI":"10.1093\/omcr\/omaa106","article-title":"Hyper IgE syndrome (Job syndrome) in Syria: A case report","volume":"2020","author":"Awad","year":"2020","journal-title":"Oxf. Med. Case Rep."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"23","DOI":"10.2500\/aap.2009.30.3193","article-title":"Elevated serum immunoglobulin E (IgE): When to suspect hyper-IgE syndrome-A 10-year pediatric tertiary care center experience","volume":"30","author":"Joshi","year":"2009","journal-title":"Allergy Asthma Proc."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"32R","DOI":"10.1203\/PDR.0b013e31819dc8c5","article-title":"Clinical manifestations, etiology, and pathogenesis of the hyper-IgE syndromes","volume":"65","author":"Freeman","year":"2009","journal-title":"Pediatr. Res."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1016\/j.jns.2004.12.012","article-title":"Chronic inflammatory demyelinating polyneuropathy in a patient with hyperIgEaemia","volume":"231","author":"Kimura","year":"2005","journal-title":"J. Neurol. Sci."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1016\/S0022-3476(03)00449-9","article-title":"Autosomal recessive hyperimmunoglobulin E syndrome: A distinct disease entity","volume":"144","author":"Renner","year":"2004","journal-title":"J. Pediatr."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1608","DOI":"10.1056\/NEJMoa073687","article-title":"STAT3 mutations in the hyper-IgE syndrome","volume":"357","author":"Holland","year":"2007","journal-title":"N. Engl. J. Med."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"864","DOI":"10.1007\/s10875-021-01051-1","article-title":"STAT3 Hyper-IgE Syndrome-an Update and Unanswered Questions","volume":"41","author":"Tsilifis","year":"2021","journal-title":"J. Clin. Immunol."},{"key":"ref_45","first-page":"180","article-title":"Hyper IgE (Job\u2032s) Syndrome: A Primary Immune Deficiency with Oral Manifestations","volume":"42","author":"Esmaeilzadeh","year":"2024","journal-title":"J. Dent. Sch."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"108988","DOI":"10.1016\/j.clim.2022.108988","article-title":"Hyper IgE syndromes: A clinical approach","volume":"237","author":"Gharehzadehshirazi","year":"2022","journal-title":"Clin. Immunol. (Orlando Fla.)"},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"e236705","DOI":"10.1136\/bcr-2020-236705","article-title":"Oral ulcerations in a patient with autosomal dominant hyper-IgE syndrome (AD-HIES)","volume":"13","author":"Borst","year":"2020","journal-title":"BMJ Case Rep."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"674","DOI":"10.18203\/2349-3291.ijcp20161064","article-title":"Hyper IgE syndrome: Often a missed diagnosis","volume":"3","author":"Singh","year":"2016","journal-title":"Int. J. Contemp. Pediatr."},{"key":"ref_49","first-page":"217","article-title":"Perforation of the nasal wall and hyper-IgE syndrome","volume":"3","author":"Fernandez","year":"1993","journal-title":"J. Investig. Allergol. Clin. Immunol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1016\/j.jaci.2024.06.022","article-title":"Allergic fungal rhinosinusitis linked to other hyper-IgE syndromes through defective T(H)17 responses","volume":"154","author":"Sun","year":"2024","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1016\/j.ijporl.2008.01.018","article-title":"Association between nasal polyposis, Dubowitz syndrome and hyper-IgE syndrome","volume":"72","author":"Sarmento","year":"2008","journal-title":"Int. J. Pediatr. Otorhinolaryngol."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"2122","DOI":"10.1111\/all.13578","article-title":"Hyper-IgE in the allergy clinic--when is it primary immunodeficiency?","volume":"73","author":"Ponsford","year":"2018","journal-title":"Allergy"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"898","DOI":"10.1080\/00365540600740470","article-title":"The clinical and laboratory survey of Iranian patients with hyper-IgE syndrome","volume":"38","author":"Moin","year":"2006","journal-title":"Scand. J. Infect. Dis."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1016\/j.revmed.2017.12.009","article-title":"Hyper-IgE in internal medicine","volume":"39","author":"Devilliers","year":"2018","journal-title":"Rev. Med. Interne"},{"key":"ref_55","doi-asserted-by":"crossref","unstructured":"Lan, J., Zhang, Y., Song, M., Cai, S., Luo, H., OuYang, R., Yang, P., Shi, X., Long, Y., and Chen, Y. (2022). Omalizumab for STAT3 Hyper-IgE Syndromes in Adulthood: A Case Report and Literature Review. Front. Med. (Lausanne), 9.","DOI":"10.3389\/fmed.2022.835257"},{"key":"ref_56","first-page":"113","article-title":"Moderate-dose intravenous immunoglobulin treatment of Job\u2032s syndrome. Case report","volume":"91","author":"Bilora","year":"2000","journal-title":"Minerva Med."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1016\/j.jaad.2005.10.022","article-title":"Hyperimmunoglobulin E syndrome: Two cases and a review of the literature","volume":"54","author":"DeWitt","year":"2006","journal-title":"J. Am. Acad. Dermatol."},{"key":"ref_58","unstructured":"Adam, M.P., Feldman, J., Mirzaa, G.M., Pagon, R.A., Wallace, S.E., and Amemiya, A. (1993). STAT3 Hyper IgE Syndrome. GeneReviews\u00ae, University of Washington."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"e1","DOI":"10.1097\/MD.0b013e31825f95b9","article-title":"Autosomal dominant STAT3 deficiency and hyper-IgE syndrome: Molecular, cellular, and clinical features from a French national survey","volume":"91","author":"Chandesris","year":"2012","journal-title":"Medicine"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1016\/j.jaci.2010.05.005","article-title":"Successful long-term immunologic reconstitution by allogeneic hematopoietic stem cell transplantation cures patients with autosomal dominant hyper-IgE syndrome","volume":"126","author":"Goussetis","year":"2010","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1007\/s10875-015-0167-z","article-title":"Successful haploidentical donor hematopoietic stem cell transplant and restoration of STAT3 function in an adolescent with autosomal dominant hyper-IgE syndrome","volume":"35","author":"Patel","year":"2015","journal-title":"J. Clin. Immunol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1007\/s10875-016-0278-1","article-title":"The Potential and Limits of Hematopoietic Stem Cell Transplantation for the Treatment of Autosomal Dominant Hyper-IgE Syndrome","volume":"36","author":"Yanagimachi","year":"2016","journal-title":"J. Clin. Immunol."},{"key":"ref_63","first-page":"S256","article-title":"Acute disseminated encephalomyelitis: An uncommon presentation of hyper IgE syndrome","volume":"24","author":"Purkait","year":"2014","journal-title":"J. Coll. Physicians Surg. Pak. JCPSP"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1097\/COH.0b013e328335ed3e","article-title":"Th17 cells, Job\u2032s syndrome and HIV: Opportunities for bacterial and fungal infections","volume":"5","author":"Milner","year":"2010","journal-title":"Curr. Opin. HIV AIDS"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1234","DOI":"10.1016\/j.jaci.2006.12.666","article-title":"Causes of death in hyper-IgE syndrome","volume":"119","author":"Freeman","year":"2007","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1177\/0003489492101S1016","article-title":"Type I allergy in the inner ear of the guinea pig","volume":"157","author":"Uno","year":"1992","journal-title":"Ann. Otol. Rhinol. Laryngol. Suppl."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1177\/019459989310800206","article-title":"Arterial supply of the human endolymphatic duct and sac","volume":"108","author":"Gadre","year":"1993","journal-title":"Otolaryngol.-Head Neck Surg."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1038\/nm1425","article-title":"Conditional ablation of Stat3 or Socs3 discloses a dual role for reactive astrocytes after spinal cord injury","volume":"12","author":"Okada","year":"2006","journal-title":"Nat. Med."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"360","DOI":"10.1016\/S0194-59989670204-8","article-title":"Allergic and immunologic aspects of Meniere\u2032s disease","volume":"114","author":"Derebery","year":"1996","journal-title":"Otolaryngol.-Head Neck Surg."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"95","DOI":"10.3109\/00016480902858899","article-title":"A comparison of three methods of using transtympanic electrocochleography for the diagnosis of Meniere\u2032s disease: Click summating potential measurements, tone burst summating potential amplitude measurements, and biasing of the summating potential using a low frequency tone","volume":"130","author":"Iseli","year":"2010","journal-title":"Acta Otolaryngol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"1202","DOI":"10.1111\/j.1398-9995.1998.tb03842.x","article-title":"Blood eosinophils and serum IgE as predictors for prognosis of interferon-gamma therapy in atopic dermatitis","volume":"53","author":"Noh","year":"1998","journal-title":"Allergy"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"73","DOI":"10.4193\/RHINOL\/21.002","article-title":"Effects on hearing and tinnitus following Dupilumab treatment of severe asthma with chronic rhinosinusitis\u2014A case report","volume":"4","author":"Thorsberger","year":"2021","journal-title":"Rhinol. Online"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"939","DOI":"10.1016\/j.jaci.2005.12.1341","article-title":"IgE memory: Persistence of antigen-specific IgE responses years after treatment of human filarial infections","volume":"117","author":"Mitre","year":"2006","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1016\/j.jaip.2013.09.013","article-title":"Antibiotic prophylaxis in primary immune deficiency disorders","volume":"1","author":"Kuruvilla","year":"2013","journal-title":"J. Allergy Clin. Immunol. Pract."},{"key":"ref_75","first-page":"1451","article-title":"Allergic cochleovestibular disturbances","volume":"76","author":"Clemis","year":"1972","journal-title":"Trans. Am. Acad. Ophthalmol. Otolaryngol."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1080\/08820139.2020.1869252","article-title":"Evaluation of Serum Immunoreactivity to Common Indigenous Iranian Inhalation and Food Allergens in Patients with Meniere\u2032s Disease","volume":"51","author":"Roomiani","year":"2022","journal-title":"Immunol. Investig."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"906","DOI":"10.1017\/S0022215111001137","article-title":"Audiological manifestations of allergic rhinitis","volume":"125","author":"Singh","year":"2011","journal-title":"J. Laryngol. Otol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1256","DOI":"10.1007\/s12070-020-02343-5","article-title":"A Prospective Study of Audiological Manifestations in Patients of Allergic Rhinitis","volume":"74","author":"Mahajan","year":"2022","journal-title":"Indian. J. Otolaryngol.-Head Neck Surg."},{"key":"ref_79","first-page":"29","article-title":"Investigation of hearing in patients with allergic rhinitis","volume":"10","author":"Karabulut","year":"2011","journal-title":"Iran. J. Allergy Asthma Immunol."},{"key":"ref_80","first-page":"653","article-title":"Use of a screening RAST in a large neuro-otologic practice","volume":"117","author":"Howard","year":"1997","journal-title":"Otolaryngol.-Head Neck Surg."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1016\/j.ebiom.2017.02.007","article-title":"Extracorporeal IgE Immunoadsorption in Allergic Asthma: Safety and Efficacy","volume":"17","author":"Lupinek","year":"2017","journal-title":"EBioMedicine"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1730","DOI":"10.1016\/j.jaci.2016.06.023","article-title":"A randomized trial of quilizumab in adults with refractory chronic spontaneous urticaria","volume":"138","author":"Harris","year":"2016","journal-title":"J. Allergy Clin. Immunol."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1080\/00016489.2019.1658898","article-title":"Intratympanically administered steroid for progressive sensorineural hearing loss in Meniere\u2032s disease","volume":"139","author":"Molnar","year":"2019","journal-title":"Acta Otolaryngol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1111\/acem.14864","article-title":"Blood biomarkers for the differentiation between central and peripheral vertigo in the emergency department: A systematic review and meta-analysis","volume":"31","author":"Klokman","year":"2024","journal-title":"Acad. Emerg. Med."},{"key":"ref_85","doi-asserted-by":"crossref","unstructured":"Zhao, Q., Sun, Y., Sun, Y., Liu, H., and Zhang, F. (2025). Upadacitinib for Treatment of Hyper-IgE Syndrome. JAMA Dermatol.","DOI":"10.1001\/jamadermatol.2025.2699"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/j.anorl.2018.03.009","article-title":"Hypereosinophilic syndrome presenting with bilateral ear fullness","volume":"136","author":"Jung","year":"2019","journal-title":"Eur. Ann. Otorhinolaryngol. Head Neck Dis."},{"key":"ref_87","doi-asserted-by":"crossref","unstructured":"Zeng, B., Domarecka, E., Kong, L., Olze, H., Scheffel, J., Monino-Romero, S., Siebenhaar, F., and Szczepek, A.J. (2024). A systematic review of the clinical evidence for an association between type I hypersensitivity and inner ear disorders. Front. Neurol., 15.","DOI":"10.3389\/fneur.2024.1378276"}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/20\/9932\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T16:29:00Z","timestamp":1760718540000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/26\/20\/9932"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,12]]},"references-count":87,"journal-issue":{"issue":"20","published-online":{"date-parts":[[2025,10]]}},"alternative-id":["ijms26209932"],"URL":"https:\/\/doi.org\/10.3390\/ijms26209932","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,12]]}}}