Auditory and Vestibular Rehabilitation In Corpus Callosum Agenesis: A Case Report and Literature Review

doi:10.21203/rs.3.rs-3235835/v1

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Auditory and Vestibular Rehabilitation In Corpus Callosum Agenesis: A Case Report and Literature Review

https://doi.org/10.21203/rs.3.rs-3235835/v1

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Background

Corpus callosum agenesis occurs when the commissural fibers between the two hemispheres cannot complete their development for various reasons. Axons from cortical areas sensitive to auditory and auditory input are transmitted from the midline through the corpus callosum. It has been shown that congenital aplasia of the corpus callosum or damage at an early age leads to disorders in the understanding of stimuli that require dichotic listening and auditory interhemispheric transmission, auditory pattern perception of sound, auditory figure, ground perception, and localization of sound. In the studies on sound lateralization, it has been revealed that compensation may be possible in cases of agenesis with corpus callosum neural plasticity and compensation mechanism. This study aims to evaluate rehabilitation, especially the auditory therapy process, and to be a reference for further studies in these cases.

Case presentation:

After the diagnosis of the corpus callosum, the patient, whose detailed examinations were made, was referred to the rehabilitation center by an external center. The 1,5-year-old patient received psychomotor, occupational therapy, and auditory therapy for 14 months during the rehabilitation process, which was applied immediately after the diagnosis process.

Conclusions

Although auditory rehabilitation is an uncommon approach in corpus callosum agenesis, the fact that the case in question has progressed in all developmental areas shows that the therapy approach is correct. This study revealed the necessity of early auditory rehabilitation in corpus callosum agenesis and laid the groundwork for future studies.

Vestibular Rehabilitation

Auditory Rehabilitation

Corpus Callosum Agenesis

The corpus callosum (CC) is the largest fiber bundle connecting the cerebral hemispheres. In this way, while providing information exchange between the two hemispheres, it plays a role in the regulation of the motor, sensory and cognitive functions of the brain. In addition, three-dimensional vision is effective in distinguishing visual information, determining the location of sound and learning processes [1]. The inability of these fibers, which have many important tasks like these, to complete their development as a result of various reasons is called corpus callosum agenesis. Many conditions that can cause agenesis have been revealed in studies. Epigenetic studies have shown that individuals with prenatal infections, maternal rubella, trisomy 8, 13, and/or 18, Andermann syndrome, Aicardi syndrome, and fetal alcohol syndrome may also have agenesis of the corpus callosum [2].

It has been shown that our lifestyles (including rehabilitative treatments) can change the way our brains function if they are of sufficient intensity and frequency. Rehabilitative therapies can be seen as a way to work neurological pathways through CNS plasticity in the long term [3].

Axons from cortical areas sensitive to auditory and auditory input are transmitted from the midline through the corpus callosum. It has been shown that congenital aplasia of the corpus callosum or damage at an early age leads to disorders in the understanding of stimuli that require auditory interhemispheric transmission, auditory pattern perception of sound, auditory figure, ground perception, and localization of sound [4].

On the other hand, the role of corpus callosum in dichotic listening has been studied, since the transfer of auditory information from one hemisphere to the other is important in auditory perception processes. Studies show that corpus callosum agenesis is associated with right ear advantage and dichotic listening, and this reveals the dominance of the left hemisphere for speech [5].

Although many studies have been done on Corpus Callosum agenesis, these studies were mostly conducted to reveal the signs of the disease. This study aims to give information about evaluation and therapy processes in these cases.

The patient was 1,5 years old, female with normal birth. It is known that the patient had a consanguineous marriage, and his elder sibling showed normal development. The patient's mother stated that the patient had frequent upper respiratory tract infections. No abnormality was found in the chromosome analysis performed on the patient in an external center. However, in the molecular genetic analysis report, an OD mutation was detected in the FREM1 gene consistent with the trigonocephaly diagnosed in the patient.

According to the information received from the family, the patient underwent a newborn hearing screening. Bilateral normal outer and middle ear structure was observed in the ENT examination performed in an external center. It was stated that the patient had a flu infection. According to the behavioral audiometer (VRA) and ABR results performed in the audiological evaluation, it was determined that the patient did not have hearing loss. Wave morphology and interwave latency were found to be normal in the ABR test.

The patient was referred to our rehabilitation center by an external center because her motor and language development was behind the normal range; she was 1,5 years old although she had no hearing loss there was no sound output and there was thought to have been a delay in her development of auditory steps and difficulty in balancing. When the patient applied to the rehabilitation center, she was evaluated in terms of physiotherapy, occupational therapy, and auditory therapy. After the first evaluation, it was noted that there was a difference between the developmental age and the chronological age of the patient. According to physiotherapy and occupational therapy evaluation; sitting without support, head control in supported sitting, crawling, object tracking, sensory problems such as feeding problems, and teeth grinding were also detected along with retardation in skills such as isolated movement patterns. As a result of the auditory evaluation made by the audiologist, it was observed that the patient needed support in auditory perception steps and sound localization.

Before the auditory rehabilitation, the patient started therapy with 3 physiotherapy sessions per week and gained the skills of sitting without support, head and body control, and crawling in these 6 months. Then, the audiologist was included in the vestibular rehabilitation process during the walking phase, and after 3 months of therapy continued in this way, the patient started walking independently.

After that, the physiotherapy sessions of the patient were completed and the sessions were continued with the same frequency to work in the field of auditory and developmental language. Along with auditory rehabilitation, basic perception steps such as recognizing the voice, distinguishing and identifying the voice, turning to the source of the voice which used lings voices, imitating it, and prolonging the joint attention span were started to be studied. At the end of the 10th month, therapy was continued with the support of occupational therapy. These sessions contributed to the patient's gaining body-environment awareness, language development, and development of joint attention.

In the 14th week of therapy, the patient's skills such as the ability to receive single instructions using only verbal cues, the ability to distinguish and localize two different sound sources, and to maintain eye contact from the beginning to the end of the speech were improved.

In language development, the patient passed the babbling period and began to give appropriate voice answers during the game. In the Denver II evaluation (2,5 years) performed at the 14

th month of therapy, there was no difference between the patient's developmental and chronological age in motor development; It was determined that the difference in language development decreased to 8 months, and the difference in social development to 10 months. The time management of the patient during the therapy process is summarized in Fig. 1.

In this study, with other areas of development auditory evaluation and therapy process of a case diagnosed with corpus callosum agenesis were presented. There are many studies in the literature about the rehabilitation process in corpus callosum agenesis. Research shows that during the rehabilitation process, specialists often focus on speech therapy, neuromotor and psychomotor therapy [3]. However, studies have shown that habilitative therapies applied early in development increase plasticity by exposing the brain to multiple stimulations [7]. In this way, it has been demonstrated that spontaneous compensation can be increased by using neural plasticity with a patient-specific therapy program in the absence of an important structure such as CC [6, 8, 14].

It was found that the rehabilitation process of 27 patients with CC agenesis was evaluated in 8 articles scanned in this study. It was determined that 16 of the patients received psychomotor therapy, 4 received language therapy and 22 received social therapy support. Looking at the scanned articles, it was seen that customized auditory therapy support is not common. The summary of the scanned articles is shown in Fig. 2. In the case presented in this study, along with occupational therapy, psychomotor and neuromotor therapy, auditory rehabilitation was also included in the patient's habilitation process.

Myelination more than doubles at 2 years of age, and this is the period when the interaction between the baby and the environment is at its highest. Completion of myelination of CC fibers occurs later than other cortical fiber tracts and may not be complete until 10 years of age or older [4].

The continuation of myelination indicates that learning is also continuing. In this respect, it has been seen that the learning process will be positively affected by starting and continuing the therapy in the early period.

In the comparison of the information coming from the two ears, the pattern perceptions of the sound are compared and the perceptual properties are analyzed and the auditory perception process continues. With the comparison of bilateral information, high-level perceptual patterns of the sound are determined and transmitted to the upper centers [4]. In this study, the auditory-perceptual processes of the case were emphasized and a therapy program was applied in this direction.

Studies have shown that dichotic listening skills and right ear advantage are affected by corpus callosum agenesis [5, 9]. However, our study found no statistical differences between the ears to the absolute latencies and interpeak in ABR. Corpus callosum agenesis has a direct effect on sound localization due to the weak connection between the hemispheres [2, 10]. In our study, all auditory perception steps were studied during the habilitation process. It is thought that this will contribute to the development of dichotic listening and has been shown to positively affect the therapy processes in other fields. Difficulty in sound localization with normal hearing has been considered a sign of a change in the auditory central nervous system [4]. In that way, occupational therapy sessions and studies for sound localization supported each other; The patient quickly gained the ability to detect the sound source.

Studies have shown the importance of following children born after the prenatal diagnosis of isolated ACC, documenting the natural course of the condition, helping children when academic or behavioral problems arise, and supporting families and caregivers [11, 12, 13].

One of the limitations of this study is that the development of the patient in question cannot be proven with a report showing the change in the neural activity of the brain. In addition, although it is the result of a general developmental evaluation, there is no criterion proving only auditory development.

Although it was a short-term habilitation process, it was revealed that the patient made progress in all areas of development as a result of multidisciplinary therapy. When compared with similar cases in the literature, it was determined that the case in question was the first patient who was taken to the auditory rehabilitation process at an early age.

It was revealed that the case discussed in our study showed improvement in all areas of development with a multidisciplinary therapy approach. In addition, the necessity of including auditory rehabilitation in the therapy process in these cases was stated and laid the groundwork for further studies.

CC: Corpus Callosum; CNS: Central Nervous System; OD: Otozomal Dominant; ENT: Ear Nose Throat; VRA: Visual reinforcement audiometry; ABR: Audıtory Braınstem Response; ACC: Agenesis of Corpus Callosum.

Acknowledgments The author thanks the patient's family and colleagues for their help and support in preparing the manuscript.

Author contributions: ZD conceived of the presented idea. OE and AÖ developed the theory. ZD, OE and AÖ investigate all aspects of this work. All authors discussed the results and contributed to the final manuscript. The author(s) read and approved the final manuscript.

Funding: No funding or sponsorship was received for this study nor for the publication of this article.

Availability of data and materials: Not applicable.

Ethics approval and consent to participate: Not required.

Consent for publication: Written informed consent was obtained from the patients' family for the publication of the case report and the accompanying images

Competing interests: The authors declare that they have no competing interests.

Patients perspective: My child has made great progress since she started rehab. She developed awareness of her surroundings and began to walk independently. I think over time it will develop much better.

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Editorial decision: Major revision
28 Feb, 2024
Reviewers agreed at journal
31 Aug, 2023
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Auditory and Vestibular Rehabilitation In Corpus Callosum Agenesis: A Case Report and Literature Review

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Abstract

Background

Case presentation:

Conclusions

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Background

Case Presentation

Discussion and Conclusions

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References

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