Related Experiment Video
Updated: Jul 3, 2026
09:44
Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss
Published on: January 25, 2016
Sensorineural hearing loss in primary antibody deficiency disorders
Marco Berlucchi1, Annarosa Soresina, Luca O Redaelli De Zinis
1Department of Pediatric Otorhinolaringology, Spedali Civili, Brescia, Italy.
The Journal of Pediatrics
|July 22, 2008
Summary
Hearing loss is common in primary antibody deficiency disorders. A significant percentage of patients with X-linked agammaglobulinemia and common variable immunodeficiency exhibited sensorineural hearing loss, necessitating regular hearing assessments.
Area of Science:
- Immunology
- Audiology
- Genetics
Background:
- Primary antibody deficiency disorders (PAD) are a group of genetic conditions characterized by impaired immunoglobulin production.
- Patients with PAD are susceptible to recurrent infections, which can potentially affect various organ systems, including the auditory system.
Purpose of the Study:
- To investigate the prevalence and characteristics of hearing impairment in patients diagnosed with primary antibody deficiency disorders.
- To determine if specific types of PAD are associated with a higher risk of hearing loss.
Main Methods:
- Audiologic evaluations were conducted on 47 patients with PAD, including 25 with X-linked agammaglobulinemia (XLA) and 22 with common variable immunodeficiency (CVID).
- Diagnostic tools included pure tone audiometry, acoustic immittance assessment, and auditory brainstem-evoked response (ABR) testing.
Main Results:
- Sensorineural hearing loss was identified in 18 out of 47 patients (38.3%).
- Hearing loss was observed in 7 patients with XLA and 11 patients with CVID.
- The hearing loss was bilateral in 12 patients and unilateral in 6 patients.
Conclusions:
- The study highlights a high frequency of hearing loss among patients with primary antibody deficiency disorders.
- Systematic audiologic screening should be integrated into the routine clinical management of individuals with XLA and CVID to ensure early detection and intervention.
Related Concept Videos
01:36Humoral Immune Responses
Overview
01:25Immunodeficiency Diseases
Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
There are three main causes of immunodeficiency disorders...
There are three main causes of immunodeficiency disorders...
01:22Development of Immunocompetence
The initiation of cell-mediated immunity can be observed as early as the third month of fetal growth, with active antibody-mediated immunity following approximately one month later.
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
01:22Myasthenia Gravis ll: Pathophysiology
The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
00:50Unrenewable Cells
In humans, the photoreceptor cells of the eye and sensory hair cells of the ear lack stem cells. These cells are thus unrenewable and cannot be replaced when they are damaged or destroyed.
Photoreceptors
The retina is composed of several layers and contains specialized cells called photoreceptors. The photoreceptors (rods and cones) change their membrane potential when stimulated by light energy. There are two types of photoreceptors—rods and cones—which differ in the shape of their outer...
Photoreceptors
The retina is composed of several layers and contains specialized cells called photoreceptors. The photoreceptors (rods and cones) change their membrane potential when stimulated by light energy. There are two types of photoreceptors—rods and cones—which differ in the shape of their outer...
01:01Hypersensitivity Reactions: Cytolytic Reactions
Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...