title: Otological findings in pediatric patients with hypogammaglobulinemia.
abstract: The main clinical presentation of patients with primary antibody deficiency (PAD) incorporates upper respiratory tract infections comprising otitis media, sinusitis and pneumonia. This study was designed to investigate clinical and paraclinical otological complications in major types of PAD. A cross sectional study was conducted on 55 PAD patients with diagnosis of selective IgA deficiency, common variable immunodeficiency (CVID), X-linked agammaglobulinemia (XLA), and hyper IgM syndrome. All patients underwent otological examinations, audiometry, and auditory brain stem response. Otological complications were detected in 54.5% of PAD patients. Conductive hearing loss was the main finding amongst PID patients (73.3%) followed by sensorineural hearing loss which was present in 8 cases. Otitis media with effusion (21.8%), chronic otitis media (27.2%), tympanosclerosis with intact tympanic membrane (5.4%) and auditory neuropathy (3.6%) were most important found complications. CVID and XLA patients with prophylactic usage of antibiotics had lower rate of audiological complications (p=0.04) and otitis media with effusion (p=0.027). As our results showed, asymptomatic otological findings were not rare in PAD patients; therefore, a systematic otological investigation is recommended as an integral part of the management and follow-up of these patients.
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran. marzieh.tavakol@yahoo.com.
authors.forename: Marzieh
authors.initials: M
authors.lastname: Tavakol
authors.affiliation: Otorhinolaryngology Research Center, Amir Alam Hospital, Department of Otolaryngology, Tehran University of Medical Sciences, Tehran, Iran. kouhi@tums.ac.ir.
authors.forename: Ali
authors.initials: A
authors.lastname: Kouhi
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran and Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at the Karolinska University Hospital Huddinge, Stockholm, Sweden. abolhassanih@yahoo.com.
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. ghajar.ar@gmail.com.
authors.forename: Alireza
authors.initials: A
authors.lastname: Ghajar
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. mhafarideh@yahoo.com.
authors.forename: Mohsen
authors.initials: M
authors.lastname: Afarideh
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. and1_sh@yahoo.com.
authors.forename: Shervin
authors.initials: S
authors.lastname: Shahinpour
authors.affiliation: Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. aghamohammadi@tums.ac.ir.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000150:complications; D001299:Audiometry; D002648:Child; D017074:Common Variable Immunodeficiency / Q000150:complications; D003430:Cross-Sectional Studies; D004427:Ear Diseases / Q000209:etiology*; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000150:complications; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D053306:Hyper-IgM Immunodeficiency Syndrome / Q000150:complications; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D008297:Male; D010033:Otitis Media / Q000209:etiology