PP1127 - Understanding Pediatric Single-Sided Deafness (SSD): Importance of Imaging in Early Childhood Case Management

Abstract:
A growing body of evidence detailing the impact of pediatric unilateral hearing loss and FDA approval for cochlear implantation in SSD patients as young as age 5 has resulted in a rise in early childhood imaging of inner ear structures. A retrospective chart review of over 100 pediatric patients with SSD reveals that approximately 50% of these patients have been identified with abnormal anatomy on the affected side. This presentation will highlight key findings from pediatric SSD data review alongside a select number of case studies to promote improved case management of infants and young children with SSD.

Summary:
Introduction
The implementation of universal newborn hearing screenings and routine hearing screenings in school-aged children has lowered the age of identification of unilateral hearing loss in pediatric patients. With FDA approval for surgery in pediatric patients with single-sided deafness as young as age 5, cochlear implantation has become an increasingly popular choice for this patient population, bringing with it a rise in early childhood imaging of inner ear structures. A retrospective chart review of over 100 pediatric patients with SSD reveals that approximately 50% of these patients have been identified with abnormal anatomy in the ear to be implanted. Of note, all cases of diagnosed unilateral auditory neuropathy spectrum disorder (ANSD) demonstrated cochlear nerve aplasia on the affected side. This presentation will highlight key findings from pediatric SSD data review alongside a select number of case studies to promote improved case management of infants and young children with SSD.

Methods
A retrospective chart review has been conducted using data available through electronic medical records (EMR). Pediatric patients with SSD were identified using referral tracking, cochlear implant evaluation patient lists, and data available in Noah. Records were reviewed for (1) abnormal anatomy as described by the radiology report, (2) cochlear implant candidacy as determined by ENT surgical consultation, and (3) intervention method as noted by the audiology report. Additionally, patient charts were reviewed for significant factors identified during case history including age of hearing loss identification and patient demographics as indicated in EMR. Several cases were additionally noted and described to highlight the benefits of early access to imaging to improve audiologic care and counseling.

Results
Thus far, a review of imaging data for 114 pediatric patients with diagnosed SSD has indicated that 62 patients were identified with abnormal anatomy, whereas only 52 patients were noted to be unremarkable. Of note, the 14 patients with diagnosed unilateral ANSD have all been identified with cochlear nerve aplasia on the affected side. In select cases of infants with identified ANSD or concern for asymmetric hearing, early access to imaging could have significantly reduced the age of final diagnosis, supported earlier access to appropriate intervention methods, and improved family counseling.

Discussion
With growing evidence to support the benefits of cochlear implantation in adults and children with SSD, detailed guidance is necessary to manage young children with unilateral hearing loss. While clinicians find agreement regarding the risks of unilateral hearing loss, recommendations and case management for infants and young children with SSD continue to be variable. Increased clinical understanding of the high risk of cochlear nerve deficiency in this population can improve counseling related to etiology and intervention options for these patients. Moreover, an earlier age of confirmation of hearing and auditory nerve status can facilitate improved case management for appropriate intervention recommendations and reduce uncertainty for families.

Brief Summary of Clinical Takeaways:
The clinical takeaway for this session is to understand the prevalence of cochlear nerve deficiency in pediatric patients with single-sided deafness and the benefits of early access to imaging.