Postural instability in children with severe inner ear malformations: Characteristics of vestibular and balance function

Abstract

Objective

To investigate the postural instability and vestibular functions in children with severe inner ear malformations (IEMs).

Design

A prospective case-control study.

Study sample

The study group consisted of 10 children using unilateral auditory brainstem implant (ABI) with labyrinthine aplasia or rudimentary otocyst. The age-matched control groups consisted of 10 unilateral cochlear implant (CI) users with normal inner ear structures and 10 healthy peers. All tests were performed to implant users when the implants were off.

Results

All median VOR gains in the ABI group (median anterior, lateral, and posterior canal 0.15, 0.05, and 0.05, respectively, for the non-implanted sides) were significantly lower than those of the control groups (median VOR gains ≥ 0.90 in both control groups). There were no oVEMP and cVEMP responses in the study group. The mean BOT-2 balance scores of the ABI (3.70 ± 1.34) group was dramatically lower than those of the CI (9.40 ± 2.88) and healthy control (16.20 ± 4.16, p < 0.001).

Conclusions

The postural instability in children with severe IEMs was higher than those in CI users with normal inner ear structures and healthy peers. The level of deficiency in the labyrinthine was more important for postural stability in children.

Keywords:

• Auditory brainstem implant
• inner ear malformation
• labyrinthine aplasia
• balance

Acknowledgements

This study has been produced from the first author’s doctoral thesis. G.E. designed and performed the study, collected data and wrote the paper; G.S. designed and consulted the study; J. K. provided statistical analysis; L.S. designed the study and analysed radiological scans. This study was approved by the Non-Interventional Clinical Researches Ethics Board of Hacettepe University, which is in accordance with the Declaration of Helsinki (GO 16/433-05).

Disclosure statement

The authors declare no conflict of interest and no funding sources for the results of this publication.