Global Data on Ear and Hearing Screening in an Intellectual Disability Population

Abstract

Intellectual disability (ID) and hearing loss are frequent comorbid conditions, although otological problems often go unnoticed until picked up by screening. In the hearing program of Special Olympics (SO), athletes with ID are screened for otological problems. By retrospective analysis of all SO meetings between 2007 and 2017, more than 100,000 screenings could be included. Cerumen impaction was found in 40.7%, middle ear problems in 29.5% of those who failed hearing screening, and hearing loss confirmation in 26.9%. Prevalences for different world regions and country income groups are provided. The results emphasize the high prevalence of hearing loss in this ID population. Awareness among health care workers and active screening are required to reduce health disparities among this disadvantaged population.

Hearing Loss Diagnosis and Management in Adults with Intellectual and Developmental Disabilities

“…SNHL is the most common type of hearing loss in the general population and is due to pathologies of the cochlea, auditory, nerve, or the central nervous system, including presbycusis, congenital syndromes, infections, ototoxicity, and trauma. In a study of 106,369 athletes aged 8–70 in the International Special Olympics between 2007 and 2017, 26.9% failed hearing screenings via distortion product otoacoustic emissions (DPOAE), tympanometry, and PTA, 67.8% of which were found to have undiagnosed SNHL [ 14 ]. Various syndromes are associated with SNHL and IDD, most commonly, DS, but also CHARGE, Hurler, Hunter, and Maroteaux-Lamy.…”

“…Various syndromes are associated with SNHL and IDD, most commonly, DS, but also CHARGE, Hurler, Hunter, and Maroteaux-Lamy. In patients with DS, SNHL is relatively low in the pediatric population, with a rate of 4.5% [ 15 ] according to one study, but increases to 65% with age [ 14 ], likely due to premature aging of the hearing system.…”

“…In contrast, CHL is due to any defect from the external ear through the ossicles impeding sound transmission to the cochlea, most commonly including cerumen impaction but also tympanic membrane perforation, otosclerosis, cholesteatomas, and chronic otitis media with effusion [ 16 ]. In the Special Olympics study described above, 32.2% of the athletes who failed screening via DPOAE were found to have conductive hearing loss [ 14 ]. In patients with DS, CHL is the most common cause of hearing loss with rates ranging from 53 to 88% [ 17 ] in children vs. 5–10% [ 18 , 19 ] in adults, which can negatively impact speech development.…”

“…While hearing loss in patients without IDD can negatively impact multiple spheres of life such as cognition, communication, social contacts, safety, and occurrence of depression, patients with IDD are even more vulnerable to these consequences, making early screening, diagnosis, and treatment vital [10]. [14]. Various syndromes are associated with SNHL and IDD, most commonly, DS, but also CHARGE, Hurler, Hunter, and Maroteaux-Lamy.…”

Evaluating the Compatibility of the Digit-in-Noise Test with Hearing Screening in Individuals with Intellectual Disabilities: A Pilot Study

“…Hearing loss is a prevalent medical condition among adults with intellectual disability (ID), affecting about 30% of this population [ 1 , 2 ]. Hearing loss can stem from various factors, such as congenital anomalies, excessive cerumen, and genetic syndromes [ 3 , 4 , 5 ]. This condition is crucial for day-to-day functioning and can impede the development of language and communication skills.…”