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Hearing Loss in Pediatric Septo-Optic Dysplasia

Brian William Herrmann1, Campbell R Hathaway2, Michael Fadell1

  • 11 Children's Hospital Colorado, Aurora, Colorado, USA.

The Annals of Otology, Rhinology, and Laryngology
|February 21, 2019
PubMed

Insights

Hearing loss affects nearly 11% of children with septo-optic dysplasia (SOD). Sensorineural hearing loss is most common, with early detection and treatment crucial for managing this condition in pediatric patients.

Area of Science:

  • Pediatric Otolaryngology
  • Neuro-ophthalmology
  • Genetics and Rare Diseases

Background:

  • Septo-optic dysplasia (SOD) is a congenital disorder characterized by the triad of optic nerve hypoplasia, pituitary abnormalities, and midline brain defects.
  • Hearing impairment is a potential, yet often under-recognized, comorbidity in children diagnosed with SOD.

Purpose of the Study:

  • To determine the prevalence and characteristics of hearing loss (HL) in a pediatric cohort with septo-optic dysplasia (SOD).
  • To identify the types of hearing loss and associated otologic conditions in children with SOD.

Main Methods:

  • Retrospective review of otologic and audiometric data for pediatric patients (<18 years) with SOD.
  • Data collected from a specialized pediatric hospital clinic (2013-2017) and supplemented by a Medline literature search.

Main Results:

  • Seven out of 64 (10.9%) pediatric SOD patients were diagnosed with hearing loss.
  • Sensorineural hearing loss (SNHL) was the predominant type (63%), often bilateral.
  • Associated findings included cochlear nerve deficiency and atresia/microtia; 57% required tympanostomy tubes.

Conclusions:

  • Nearly 11% of children with SOD experience hearing loss, primarily SNHL.
  • This study is the first to report on hearing loss in a pediatric SOD cohort, including novel findings of cochlear nerve deficiency and atresia/microtia.
  • Early identification of hearing loss via audiological evaluation and imaging, alongside prompt otitis management, is recommended for this population.
Abstract

Keywords:
hearing losspediatricsepto-optic dysplasia

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