Unimodal versus bimodal auditory stimulation in inner ear malformations: Cognitive, language, and motor performance.

Purpose: New perspectives on rehabilitation options for inner ear malformations have still been studied in the literature. This study investigated the cognitive, language, and motor skills of auditory brainstem implant (ABI) users in unimodal and bimodal groups. Methods: The motor competency of the participants was assessed with Bruininks-Oseretsky Motor Proficiency Test-2 Short Form (BOT2 SF). Language performance was evaluated by the test of Early Language Development-3 and Speech Intelligibility Rating. Word identification, sentence recognition tests, and Categories of Auditory Performance were used to assess auditory perception skills. To examine the cognitive performance, Cancellation Test and Gesell Copy Form were administered. All the tests were conducted in a quiet environment without any distractions. Results: The participants were divided into two groups: (1) 17 children in the unimodal group and (2) 11 children in the bimodal (who used a cochlear implant on one side and ABI on the other side) group. There were significant correlations between the chronological age of participants and BOT2 SF total score, cancellation tests, auditory perception tests, and language performance. Similarly, there were significant correlations between the duration of ABI use and auditory perception tests, language performance, cancellation test, and some BOT2 SF subtests (r = -0.47 to -0.60, p < .001). There was no significant difference between the unimodal and bimodal groups in any task (p > .05). However, there were moderate-to-strong correlations among the auditory perception tests, cancellation test, language test, and BOT2 SF total score and subtests (r = 0.40 to 0.55, p < .05). Conclusion: Although there were no significant differences between bimodal and unimodal groups, a holistic approach, which indicates that hearing and balance issues can have broader impacts on a person's physical, emotional, social, and psychological aspects, should be used in the assessment process. Level of Evidence: Level 4.

Clinical use of child-friendly video head impulse test in dizzy children.

OBJECTIVE: To investigate the high-frequency vestibular functions using a child-friendly video head impulse test and to classify vestibular symptoms using a standard nomenclature guideline in dizzy children. MATERIALS AND METHODS: The video head impulse test with a remote camera was used in this study. The data of 40 dizzy children (7.30 ± 4.75 years) were analyzed retrospectively. RESULTS: The high-frequency vestibulo-ocular reflex gains of 14 (35%) dizzy children in the study group were reduced, whereas the vestibulo-ocular reflex gains of 26 (65%) dizzy children were normal. Two of them had a dramatic reduction in vestibulo-ocular reflex gains on their affected SCCs, but no corrective saccades as their gazes had not been fixated on the target at the onset of head impulses. In dizzy children, the most common cause was inner ear malformation (15%) and the most common vestibular symptom was unsteadiness (25%). CONCLUSION: The high-frequency vestibular function of the VOR in dizzy children, especially those suffering from IEM and unsteadiness, can be quickly screened using the vHIT device with the remote camera. However, clinicians should be careful during the interpretation of traces in the pediatric group.

Postural Control in Subjects with Incomplete Partition Inner Ear Malformations: A Comparison of Incomplete Partition Types.

INTRODUCTION: Children with inner ear malformation (IEM) are at risk of vestibular loss as well as hearing loss. Incomplete partition (IP) anomalies constitute about 41% of all IEMs. This study aimed to investigate the postural control in subjects with the same type of IP on both sides and to compare their results with cochlear implant (CI) users without IEM and healthy peers. METHODS: The study group consists of 17 subjects with the same IP types on both sides and using auditory implants on at least one side, with the following 3 groups: 6 IP-I subjects (mean age 12.28 ± 6.25), 6 IP-II subjects (mean age 12.90 ± 3.23), and 5 IP-III subjects (mean age 6.98 ± 3.10). Six unilateral CI users (mean age 11.38 ± 3.57) with normal inner ear structures were included in the CI control group, and 6 healthy peers (10.20 ± 4.79) were included in the healthy control group. The postural control was measured using the Bruininks-Oseretsky Test of Motor Proficiency Second Edition (BOT-2) balance subtest. All devices were turned off during the balance test. RESULTS: The BOT-2 balance scale scores were observed to be significantly different between the IP-I and healthy control group (medians of balance scores being 3.00 and 16.00, respectively, p < 0.001) and the IP-III and healthy control group (medians of balance scores being 6.60 and 16.00, respectively, p = 0.04). The IP-II group had better balance scores (median = 8.00) than those of the other IP groups, although there were no significant differences between the IP-II and other groups (p > 0.05). CONCLUSION: This study demonstrated that subjects with the same IP type on both sides and with early implantation may differ in terms of their postural control abilities depending on their IP type. Subjects with IP should be regularly followed up by the vestibular assessment and supported by their postural control ability by vestibular rehabilitation.

Impairment of vestibulo-collic reflex and linear vestibulo-ocular reflex in pediatric-onset multiple sclerosis patients.

OBJECTIVES: This study aimed to examine the vestibulo-collic reflex (VCR) and linear vestibulo-ocular reflex (lVOR) and their correlation with brain lesions in pediatric-onset multiple sclerosis (POMS). METHODS: The study group consisted of 17 patients (34 ears) with POMS (mean age 18.73 ±â€¯2.02, mean age at disease onset 14.64 ±â€¯1.36 years), and the control group included 11 age-matched healthy subjects (22 ears). Ocular and cervical Vestibular Evoked Myogenic Potentials (oVEMP and cVEMP) were performed to assess IVOR and VCR pathways. Magnetic Resonance Imaging was evaluated in the study group. RESULTS: In the POMS group, 47.05 % of oVEMPs and 17.64 % of the cVEMPs were abnormal, while all VEMPs were normal in the control group. The oVEMP amplitude was associated with infratentorial lesion volume (r = -0.459, p = 0.01) and total lesion volume of the brainstem and cerebellum (r = -0.450, p = 0.01). The cVEMP asymmetry ratio was correlated with the deep white matter lesion volume (r = 0.683, p < 0.001). The MVEMP scores were found to correlate only with lesion volumes in the cerebellum (r = 0.488, p = 0.04) and infratentorial region (r = 0.573, p = 0.01). CONCLUSIONS: Ocular and cervical VEMP abnormalities confirm that lVOR and VCR pathways may be affected in early POMS. SIGNIFICANCE: Routine use of the VEMP test, especially the oVEMP test is recommended as a useful tool in the follow-up of POMS patients.

Children with Auditory Brainstem Implant: How Do They Perform in Motor and Language Skills?

BACKGROUND: Young children are able to explore new objects and practice language through the acquisition of motor skills that lead to their overall development. Congenital hearing loss and total vestibular loss may contribute to the delay in speech and motor skill development. OBJECTIVES: To investigate the relationship between motor development performance, speech perception, and language performance in children with auditory brainstem implant (ABI). METHOD: Ten children, aged 4-17 years (mean age 9.76 ± 4.03), fitted with unilateral ABI for at least 2 years due to the presence of labyrinthine aplasia and rudimentary otocyst at least 1 side were included in the study. Several standardized tests, such as Bruininks-Oseretsky Motor Proficiency Test-2 (BOT-2), Children's Auditory Perception Test Battery, Meaningful Auditory Integration Scale (MAIS), and Test of Early Language Development-3, were performed to evaluate their skills of fine motor control, balance, manual dexterity, language, and auditory perception. RESULTS: A significant correlation was established between the BOT-2 manual dexterity and MAIS scores (r = 0.827, p < 0.05) and between the manual dexterity and language skills (for expressive language, r = 0.762, p < 0.05; for receptive language, r = 0.650, p < 0.05). Some of the BOT-2 balance tasks, such as standing on 1 leg on a line with eyes closed, standing on 1 leg on a balance beam with eyes open, standing heel-to-toe on a balance beam, and walking forward heel-to-toe on a line, showed a strong correlation with their receptive and expressive language performance (p < 0.05). CONCLUSION: The current study has indicated that significantly poor manual and balance performances are associated with poor speech perception and language skills in children with ABI. The authors recommend performing a vestibular assessment before and after ABI surgery and the use of a holistic rehabilitation approach, including auditory and vestibular rehabilitation, to support development of the children with ABI.

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

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.

Vestibulo-ocular reflex involvement in childhood-onset multiple sclerosis.

BACKGROUND: Multiple Sclerosis (MS), an autoimmune demyelinating disease of the central nervous system, is an important cause of disability in young adults. The purpose of this cross-sectional study was to evaluate the vestibular system with video Head Impulse Test (vHIT) and determine the impairment of the Vestibulo-ocular Reflex (VOR) in childhood-onset MS. METHODS: The study group, 20 persons with MS (pwMS) with onset before 18 years of age (6 M, 14 F; mean age 19.06 ± 1.66) and the control group, 20 healthy, age- and sex-matched individuals were retrieved from vHIT recordings. The mean age of MS onset in the study group was 14.60 ± 1.53 years. The VOR pathway was evaluated using vHIT. RESULTS: The median VOR gains of right anterior (1.00), left lateral (0.96) and left posterior (0.91) semicircular canals were significantly lower in the pwMS group than those of the healthy control group (1.05, 1.00, 0.98 respectively, p < 0.05). Four of pwMS (20%) had abnormal VOR gains. The pwMS with dizziness had significantly lower VOR gains (median 0.91) compared with pwMS without dizziness (median 1.01, p < 0.05). CONCLUSION: This study demonstrates vestibulo-ocular system can be affected in patients with childhood-onset MS and suggests using vHIT especially in the follow-up of pwMS with dizziness.