Sam’s incredible journey: A case of cerebellar ataxia Author: Maude Le Roux Contact: maude@atotalapproach.com Center: A Total Approach Country: United States (Glen Mills, Pennsylvania) Month: July Year: 2008 Abstract: This report describes the case of a young boy with a medical diagnosis of cerebellar ataxia who progressed from needing a wheelchair to walking with handheld assist after Tomatis Training was added to a program of occupational and physical therapy.The boy had previously been discharged from an extensive therapy program for not making sufficient progress.The consultant concludes that adding Tomatis Training to a two-hour program of occupational and physical therapy was responsible for the remarkable progress achieved. Keywords: cerebellar ataxia, Tomatis Method, occupational therapy
Cerebellar ataxia refers to a condition of unsteadiness of gait. Causes of ataxia are varied. It may be caused, for example, by meningitis as in the case of Sam. Sam had an unspecified diagnosis of either bacterial or viral meningitis. Bacterial meningitis is a serious infection of the fluid in the spinal cord and the fluid that surrounds the brain. It is most commonly caused by one of three types of bacteria: Haemophilus influenzae type b, Neisseria meningitidis, and Streptococcus pneumonia bacteria. Bacterial meningitis is considered more serious than viral meningitis as it can result in brain damage and even death. Viral meningitis is a relatively common, but rarely serious, infection affecting the fluid in the spinal cord and the fluid that surrounds the brain as well. Viral meningitis is caused by any of a number of different viruses, many of which are associated with other diseases. Mosquito-borne viruses can also cause viral meningitis. There is no specific treatment for viral meningitis, which is usually mild and clears up in a week. BACKGROUND Sam, a young boy, born in 1994, experienced an uncomplicated early childhood, played lacrosse, basketball, and soccer. He contracted meningitis in September 2005, at the age of 10 years, 11 months. Hospitalized from September 24, 2005, through October 4, 2005, Sam later attended a rehabilitation center in Pennsylvania, USA, where he was diagnosed with resultant cerebellar ataxia. Sam was treated as an outpatient from October 2005 through March 2006, when he transitioned to our program. After being discharged from the rehabilitation services (6 hours weekly including occupational therapy and physical therapy services), Sam was seen for a joint occupational and physical therapy evaluation at our center, A Total Approach, on March 15, 2006.
INITIAL EVALUATION At the time of the initial evaluation, Sam was wearing a scopolamine patch for his consistent experience of vertigo and nausea. He required total assistance for toileting skills, though he was able to verbalize when he needed to go. He was being homeschooled through his local school district and was wheelchair-bound due to an unstable gait pattern with severe balance impediment. He experienced difficulty holding any utensils, handwriting, as well as manipulating any objects. His hands became shaky when contemplating fine-motor tasks. Sam used a 4-digit pattern loose grasp on a pencil, decreased web space, and decreased ulnar (one of the fore-arm bones) stabilization, using isolation at his elbow as a compensation for flexibility at his wrist and digits. He rubbed his eyes after visual tasks and experienced blurred vision after sustained reading. Sam was unable to stand without support, walked with a two-hand assist or walker, fell easily, experienced difficulty throwing and catching, carrying objects, cutting, and pouring. He demonstrated poor trunk control overall and became fatigued quickly after motor output. Sam was able to communicate very well, continued to maintain a number of friends, could control his frustration, was fully aware of social situations, and was generally in good humor. He was independent in upper-body dressing, but required moderate assistance in lower-body dressing. He showered while seated on a tub bench and needed help cutting and preparing his food. CONSULTANT’S ANALYSIS Dr. Alfred Tomatis (1991) believed that the vestibular system, which was primarily affected in Sam, was an integral part of the entire central nervous system. We also know from neuroscience (Castro et al., 2002) that auditory and vestibular system information is processed through the 8th cranial nerve, the vestibular-cochlear nerve, and is largely connected to the impact it has on the cerebellum. Sam was struggling intensely to find a sense of balance, a sense of being centered, and we needed to work on laterality through multiple systems, the integration of these systems being essential to making a difference in his functional capacities. INTERVENTION Sam completed three loops of Tomatis Training in combination with occupational and physical therapy exercises targeted specifically toward his unstable gait patterns, different gross motor transitions (sit-to-stand, four point kneeling, half kneeling, etc.), and also targeted toward the fine motor skills. Though some of the activities would have been standard practice procedures, similar to therapies that were completed previously at the rehabilitation center, other activities were added that would be considered to be more characteristic of the field of Sensory Integration. In Sensory Integration, we believe in working on all sensory systems together to create an effective adaptive response within the central nervous system. The first intensive loop of 15 days included no filtered words, and it was decided to use a very gradual process of changing the precession and delay* of the Electronic Ear* to be at about 5 and 50 on the last day. We started shifting the balance toward the right ear on the eighth day of Sam’s first loop; we introduced filters gradually from Day 5. After the first day in Tomatis Training, Sam commented to his mother that “his legs felt lighter.” After Day 3, he seemed to have more energy and was able to walk 500 feet twice with his walker at home. By Day 5, his mother commented that he appeared to have a steadier gait. After Day 11, Sam noted that he was now able to stop himself
from falling, when he started to fall. On Day 12, Sam’s mother commented that he was now walking around the house independently without his walker. By the end of the 15-day loop, Sam was no longer using the wheelchair. Sam was in his first week of a four-week break period when we received a frantic call from his father. During the first loop, we had decided to leave on the vestibular patch as it was a medical intervention, and we did not feel comfortable making the decision to remove it. Because Sam was undergoing so many tremendous changes, his parents decided to remove the patch at their own risk. Sam seemed to have taken a turn for the worse and was unstable again, feeling very nauseated. We recommended that the family go to their physician, and we gave them a therapeutic CD of modulated music of Mozart, a system designed by occupational therapist Sheila Frick. [1] Within two days, Sam was back on track and without his vestibular patch, needing no more of this medical support. The parents reported this episode to their doctor only at a follow-up visit after the completion of Tomatis Training. During the second loop (eight days), we focused more on the lower sound frequencies with smaller bursts into the higher frequencies and used the SBe gradual process on precession and delay; on Day 3 we started to work more vigorously on laterality. Sam’s profile continued to progress gradually; he started walking more independently on outside unstable surfaces without his walker. His active reading into the microphone, introduced on the third day, also improved steadily with each day. By Loop 3 (another eight days), the focus was completely shifted to walking. Sam’s gait pattern was irregular, and he required handheld assist to walk without his walker. His stability and fluidity were improving steadily day after day. We used higher frequencies more vigorously during this loop and, by the end of the loop, Sam was walking with handheld assist over unstable surfaces inside and outside of buildings. RESULTS OF THE TOMATIS LISTENING TEST Sam’s Tomatis Listening Test (TLT) taken before the first loop of Tomatis Training, indicated a superimposed bone conduction pattern in his left ear, with an underactive air conduction curve. A similar pattern was observed in his right ear with the exception of a mingling of the two curves around 1500 Hz. Sam reported hearing “ringing” in his right ear and exhibited several spatialization errors* in his right ear. The TLT administered when he returned for his second loop showed the air conduction on both ears superimposing the bone conduction in the lower frequencies, with intermingling in the mid frequencies. Bone conduction continued to superimpose in the higher frequencies. No more spatialization errors were noted. Post testing, after completion of his third loop, indicated intermingling of the air and bone conduction curves in both left and right ears, although there was still some superimposing of the bone over the air curve in the very high frequencies. According to Sam, the ringing in his right ear was “more silent” by the end of the third loop, and he did not complain of ringing in his right ear again. The functional results of the Tomatis Program have been reported above. The success of the more natural air conduction curves in both ears in the lower and mid frequencies correlated very well with the motor changes we observed during Sam’s treatment period.
CONCLUSION Sam came to Tomatis Training after having undergone much intensive therapy based on a diagnosis of cerebellar ataxia. He arrived with a very unstable gait pattern, needing a wheelchair, and walking with a walker only when supervised. At the time, Sam had very little sense of laterality, and his position related to center of gravity; he wore a vestibular patch to prevent nausea. When he exited our program after three loops of Tomatis Training, he was walking with handheld assist and with no need for the wheelchair. Sam played lacrosse before the onset of his illness, and one of his goals was to be able to string the head of his lacrosse stick again, which he was able to do, though at a slow pace. He was eating and writing with utensils, and he did not experience the blurriness of vision during reading at this time. Since Sam had experienced multiple occupational and physical therapies intensively on a daily basis prior to coming to our center, we have to conclude that what made the real difference in this young man’s life was the Tomatis Program. Our results with Sam support Tomatis’s belief that the vestibular system is integral to the functioning of the central nervous system (Sollier, 2005). Given that these results demonstrate the robustness of the Tomatis Method of sound stimulation as a means of strengthening balance and coordination in the context of a complex condition such as cerebellar ataxia, we recommend that Tomatis Training be added to the traditional treatment options (Perlman, 2000) for this condition. REFERENCES •
• • • • • • • •
Boon, R. (2000, May). Central auditory processing disorder: From central auditory processing skills to language and literacy. Paper presented at Speech Pathology Australia, National Conference, Adelaide. Article retrieved from http://home.iprimus.com.au/rboon/CAPD.htm. Butler, K. (1980). Disorders of other aspects of auditory function. In R. VanHattum (Ed.), Communication disorders (pp. 123-158). New York: Macmillan Publishing. Katz, J. (1992) Classification of auditory processing disorders. In J. Katz, N. A. Stecker, & D. Henderson (Eds.) Central auditory processing: A transdiciplinary view (pp. 81-92). St. Louis: Mosby Press. Lerner, J.A (1990). Phonological awareness: A critical element in learning to read. Learning Disabilities: A Multidisciplinary Journal. 1(2), 72 - 76. Levinson, P., & Sloan, C. (Eds.). (1980). Auditory processing and language: Clinical and research perspectives. New York: Grune & Stratton. Rampp, D. (1980). Auditory processing and learning disabilities. Lincoln, NE: Cliff Notes, Inc. Tomatis, A. A. (1963/1978). L’Oreille et le language. Paris: Éditions du Seuil. Tomatis, A. A. (1996). The ear and language. (B. Thompson, Ed. and Trans.). Norval, Ontario: Moulin Publishing. (Original published in 1963 in French) Willeford, J. A., & Burleigh, J. M. (1985). Handbook of central auditory processing disorders in children. Orlando, FL: Grune & Stratton.
FOOTNOTES 1. The study by Hall and Case-Smith (2007) listed in the References involved the use of the Therapeutic Listening Program designed by Sheila M. Frick, OTR. More information on her work can be obtained at www.vitallinks.net.