Hearing loss with cochlear modiolar defects and large vestibular aqueducts

Am J Otol. 1998 May;19(3):306-12.


Objective: Defects of the cochlear modiolus have been found to be associated with most cases of large vestibular aqueduct. The clinical significance of these modiolar defects has not been studied previously. The purpose of this article is to correlate clinical (functional) parameters, such as hearing outcomes, with the severity of the radiographic findings in these dysplastic inner ears.

Study design: The study design was a retrospective chart review, supplemented with telephone interviews and clinic visits.

Setting: The study was conducted at an academic, tertiary care center.

Patients: Thirty consecutive patients with large vestibular aqueducts participated.

Results: Scores of modiolar deficiencies yielded inconsistent correlations with hearing loss. Vestibular aqueduct morphology and thickness correlated very strongly with the severity of hearing loss.

Conclusions: These observations support the hypothesis that large vestibular aqueduct-related hearing loss may be caused by transmission of subarachnoid pressure forces into the inner ear. However, the thickness and morphology of the vestibular aqueduct may simply be markers for more subtle cochlear dysplasia manifest by modiolar deficiency.

MeSH terms

  • Adolescent
  • Adult
  • Auditory Threshold
  • Child
  • Child, Preschool
  • Cochlea / abnormalities*
  • Cognition Disorders / etiology
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Hearing Loss, Conductive / diagnosis*
  • Hearing Loss, Conductive / etiology*
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Retrospective Studies
  • Speech Reception Threshold Test
  • Vestibular Aqueduct / abnormalities*