Hearing impairment is common in patients with mucopolysaccharidoses (MPS) in the preschool age. Conductive or mixed hearing loss is the most frequent occurrence while the involvement of the inner ear or central auditory pathways may occur in more severe forms. A retrospective review of 82 children with MPS admitted at the Pediatric Department of the University of Milano Bicocca was performed to determine the incidence of otological symptoms. We focused particularly on audiological investigations in a subgroup of 47 children diagnosed before 6 years of age (MPS I, n = 11 patients; MPS II, n = 10; MPS III, n = 7; MPS IV, n = 14; MPS VI, n = 5). In 37 children, a magnetic resonance imaging (MRI) of the brain and cervical spine was also performed in order to correlate the audiological findings with the imaging of the middle and inner ear. A total of 40 out of 47 children (86%) showed some degree of hearing impairment: sensorineural or mixed hearing loss in 23 cases (48.93%) and retrocochlear in 4 (8.51%). MRI ascertained multiple CNS abnormalities in 13 (35.3%): dilated perivascular spaces in 5 (38.5%); dilated ventricular cavities in 5 (38.5%); demyelinated and gliotic areas in 3 (23.0%). Conversely, one-fourth of the children’s inner ears showed some morphological anomaly (24.3%).
Part of the book: An Excursus into Hearing Loss
The last decade has witnessed significant advances in imaging of the middle and inner ear and the auditory pathways. High resolution computerized tomography (CT) scanners and new magnetic resonance (MR) sequences have been implemented in clinical practice as valuable supportive tools for the Audiologist in the identification of the site of lesion and for the surgical planning by the Otologist. The purpose of this chapter is to review the current advanced methods of neuroradiological evaluation of patients with sensorineural hearing loss (SNHL), either congenital or acquired, especially focusing on the assessment of candidates to cochlear implantation (CI), with plenty of explicative images.
Part of the book: Advances in Rehabilitation of Hearing Loss