Hearing Loss in Pediatric Septo-Optic Dysplasia

Objective: To identify and characterize hearing loss (HL) in children with septo-optic dysplasia (SOD). Methods: Otologic and audiometric data for patients less than 18 years of age identified as having SOD who were seen in the Children's Healthcare of Atlanta-Scottish Rite Hospital clinic between 2013 and 2017 were collected and reviewed through a HIPAA-compliant medical record search. Relevant literature was also reviewed with the assistance of Medline. Results: Sixty-four patients with SOD were identified, and 7 of those patients (10.9%) were diagnosed with hearing loss. Type of hearing loss was sensorineural (SNHL) in 5 patients (63%), mixed (MHL) in 1(14%), and conductive (CHL) in 1(14%). Bilateral loss presented in 60% (3/5) of SNHL patients, while the rest demonstrated unilateral loss. Unilateral findings included cochlear nerve deficiency (1) and atresia/microtia (1). Tympanostomy tubes were required in 57% (4/7) of SOD children with hearing loss. Amplification was successfully implemented in 86% (6/7). Conclusions: Hearing loss was found in nearly 11% of SOD children, and SNHL was identified as (63%) the predominant form of loss. To our knowledge, this is the first retrospective review of hearing loss in a pediatric SOD cohort and the first to report of cochlear nerve deficiency and atresia/microtia in this population. Based on these findings, early identification of hearing loss with imaging when appropriate and treatment of otitis in this population is recommended.