Abstract
The occurrence of unilateral sensorineural hearing loss (SNHL) during school age is relatively rare and accounts for approximately 6% of all deafness in childhood. We present two cases involving children who were diagnosed with SNHL associated with Sjögren's syndrome (SS). Case 1: An eight-year-old girl with an approximately two-year clinical history of gradual hearing loss was diagnosed with SNHL associated with SS based on histological findings of inflammation in the salivary glands and the presence of serum anti-Sjögren's syndrome-A antibody. Case 2: An eight-year-old boy with acute idiopathic thrombocytopenic purpura in whom unilateral hearing loss, which was not associated with any problems in daily life, was detected during hospitalization and who was finally diagnosed with SNHL and SS. Steroid treatment was ineffective for both patients. The previously unrecognized combination of SNHL with SS should be considered in the diagnosis of unilateral SNHL, even in children.
Highlights
Congenital sensorineural hearing loss (SNHL) is one of the most common congenital disorders [1], with a prevalence of one to two per thousand live births; its major etiologies include genetic causes, cytomegalovirus (CMV) infection (21%), and other environmental causes (14%) [2]
Most patients with SNHL are identified by newborn hearing screening with an automated auditory brainstem response (AABR) examination during the neonatal period or by routine health check-ups for preschool children
Steroid treatment was started on the 19th day of admission, and tapered off after 18 days. His platelet level successfully remained at >50,000/μL; no remarkable change in hearing activity in his left ear was observed by ABR or distortion product otoacoustic emissions (DPOAEs) in the three months after the treatment. These two children, in whom unilateral hearing loss was noticed at school age, were diagnosed with unilateral SNHL associated with s syndrome (SS)
Summary
Congenital sensorineural hearing loss (SNHL) is one of the most common congenital disorders [1], with a prevalence of one to two per thousand live births; its major etiologies include genetic causes (approximately two-thirds), cytomegalovirus (CMV) infection (21%), and other environmental causes (14%) [2]. Temporal bone CT and MRI showed no structural abnormalities in the inner ears (Figures 10-12) His serum test findings were as follows: anti-CMV IgG antibody, 106 AU/mL (cutoff,
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
