Developmental Delay … Or Something Else? A boy’s school struggles lead to central auditory assessment—and an unexpected, but crucial, finding. Case Puzzler
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Case Puzzler  |   July 01, 2016
Developmental Delay … Or Something Else?
Author Notes
  • Jeanane M. Ferre, PhD, CCC-A, is an audiologist in private practice in Oak Park, Illinois. She is an adjunct faculty member at Northwestern and Rush universities and chairs the coordinating committee of ASHA Special Interest Group 6, Hearing and Hearing Disorders: Research and Diagnostics. jmfphd@comcast.net
    Jeanane M. Ferre, PhD, CCC-A, is an audiologist in private practice in Oak Park, Illinois. She is an adjunct faculty member at Northwestern and Rush universities and chairs the coordinating committee of ASHA Special Interest Group 6, Hearing and Hearing Disorders: Research and Diagnostics. jmfphd@comcast.net×
Article Information
Special Populations / School-Based Settings / Case Puzzler
Case Puzzler   |   July 01, 2016
Developmental Delay … Or Something Else?
The ASHA Leader, July 2016, Vol. 21, 38-40. doi:10.1044/leader.CP.21072016.38
The ASHA Leader, July 2016, Vol. 21, 38-40. doi:10.1044/leader.CP.21072016.38
Dylan, a second-grader, came to me for a central auditory evaluation through a referral from his neuropsychologist. Dylan had been diagnosed during early childhood with visual impairment due to cranial nerve palsy involving the right oculomotor nerve (III) that resulted in drooping of the upper eyelid (ptosis) and restricted eye movement.
At just 7 years old, he’d already undergone two eye surgeries and was receiving vision therapy. He was struggling at school with reading, speech-language, listening and sensorimotor skills—struggles attributed to developmental delay and his visual impairment.
But his neuropsychologist suspected possible auditory processing impairment as a cause for his language and learning difficulties. His parents reported that he had a febrile seizure at 13 months secondary to viral meningitis, with no reported abnormalities on a CT scan done at that time.

Dylan’s ability to recognize speech presented to his right ear—with or without a competing left-ear signal—was significantly poorer than for left-ear presentations.

The central auditory evaluation consisted of standard puretone and speech audiometrics, immittance testing, and administration of low redundancy speech tests, dichotic listening tests and temporal patterning tests. Results showed that Dylan had normal hearing sensitivity bilaterally. But his ability to recognize speech presented to his right ear—with or without a competing left-ear signal—was significantly poorer than for left-ear presentations (see sidebar below for his test results).
But why? This wasn’t a peripheral hearing loss, and it wasn’t the kind of central auditory impairment that I was used to seeing. Dylan needed help with his auditory-language skills. But, without more information, what form should that help take? I formulated two hypotheses.
The first was that, given his history of right oculomotor palsy, he had another undiagnosed palsy affecting his right auditory nerve. (No brain imaging studies had been done when the oculomotor palsy was initially diagnosed.) My second hypothesis was that Dylan’s symptoms resulted from a dysfunction of the left (contralateral) auditory cortex.

This wasn’t a peripheral hearing loss, and it wasn’t the kind of central auditory impairment that I was used to seeing.

An unexpected finding
To investigate these hypotheses, I referred Dylan for follow-up auditory evoked potential testing. Although most of the findings were normal, the testing detected bilateral deterioration of Wave V with increased click rate. Wave V abnormalities with increased click rate have been reported among patients with multiple sclerosis (see sources below). And, although uncommon, multiple sclerosis has been linked to oculomotor palsy (see sources). Based on these findings, Dylan’s neurologist referred him for an MRI scan.
The MRI revealed volume loss of the left temporal lobe consistent with cystic encephalomalacia, a softening of neurologic tissue within a cystic cavity. This type of condition is typically caused by insufficient blood supply, often following a traumatic brain injury. Dylan’s neurologist believed that his trauma had been the early childhood meningitis, and his condition had “developed around” the insult.
The effect of the encephalomalacia was significant. Imaging revealed abnormality to most of the left temporal lobe, including most of the left hippocampus and left amygdala.
When I consulted with Dylan’s neurologist, he suggested that Dylan’s right auditory cortex had been doing—and continued to do—“most of the auditory work.” As evidence, he pointed to the dramatic difference between Dylan’s right and left ear scores across most central auditory processing tests.

Our plan for Dylan, his teacher and his classroom included environmental modifications and accommodations that would enhance signal quality and signal-to-noise ratio reaching the ear.

What now?
There is no known treatment for cystic encephalomalacia, as dead tissue cannot be made to work again. Research continues to explore the extent to which normal function can be restored—indications are that it’s likely related to the extent of tissue involvement.
In Dylan’s case, we focused our intervention on improving his auditory functioning. That is, we opted to manage his day-to-day listening needs the way we would a student with a unilateral hearing loss. Our plan for him, his teacher and his classroom included environmental modifications and accommodations that would enhance signal quality and signal-to-noise ratio reaching the ear.
For Dylan:
  • Sit/stand near and facing the speaker.

  • Use visual cues as much as possible—look and listen.

  • Wait for all instructions before beginning.

  • Listen for meaning. Guess when possible.

  • Ask for repetition or clarification as needed.

  • If excessive noise is present, direct noise toward right (poorer) ear with target toward left (better) ear.

For the instructor:
  • Gain all students’ attention before giving instructions.

  • Use clear speech (speaking slightly slower and slightly louder).

  • Repeat information as needed with associated visual cue and/or demonstration.

  • When giving multistep directions, alert the listener to the total number of steps (for example, “I want you to do three things”) then “tag” items with words such as first, last, before and after, and pause briefly (one or two seconds) between items to enhance processing.

  • Allow “thinking time” before expecting a response.

For the school:
  • Provide advance notice of material and opportunity to become familiar with task demands, vocabulary and general concepts before diving in to a task (for example, provide a study guide at the outset of a unit rather than the day before a test).

  • Limit oral exams.

  • Provide extended time for all examinations, including high-stakes exams.

  • Provide listening breaks throughout the day to minimize auditory fatigue.

  • Initiate trial use of an assistive listening device in the classroom.

Stay tuned
Dylan’s diagnosis suggests limited, at best, results of any auditory training to enhance his auditory skills. However, his parents were interested in implementing aural rehabilitation, including auditory training, as part of his IEP. His school-based speech-language pathologist is providing intervention to improve Dylan’s language, functional communication and listening skills. Dylan will also receive home-based computer-assisted auditory training with targets directed to his right ear only. After 30 hours of this training, he will return to the office for a recheck of his central auditory test performance. Time will tell if we see improvement.
Most children presenting with symptoms of central auditory processing impairment do so for reasons other than demonstrable neurologic dysfunction—that is, they have delayed development of these skills rather than identifiable neurologic impairment. Dylan’s case reminds us that our ability to use auditory information is neurologically based. It further reminds us that, even among children, central auditory processing tests can be powerful tools—not only to clarify children’s listening and communication challenges, but also to help with the differential diagnosis of the conditions underlying those challenges so we can provide effective intervention.
Figure 1.

Dylan’s Auditory Test Results

Dylan’s Auditory Test Results
Figure 1.

Dylan’s Auditory Test Results

×
Sources
Bhatti, M. T., Schmalfuss, I. M., Williams, L. S., & Quisling, R. G. (2003). Peripheral third cranial nerve enhancement in multiple sclerosis. American Journal of Neuroradiology, 24, 1390–1395. [PubMed]
Bhatti, M. T., Schmalfuss, I. M., Williams, L. S., & Quisling, R. G. (2003). Peripheral third cranial nerve enhancement in multiple sclerosis. American Journal of Neuroradiology, 24, 1390–1395. [PubMed]×
Levine, R. A., Gardner, J. C., Stufflebeam, S. M., Fullerton, B. C., Carlisle, E. W., Furst, M., . . . Kiang, N. Y. (1993). Binaural auditory processing in multiple sclerosis subjects. Hearing Research, 68(1), 59–72. [Article] [PubMed]
Levine, R. A., Gardner, J. C., Stufflebeam, S. M., Fullerton, B. C., Carlisle, E. W., Furst, M., . . . Kiang, N. Y. (1993). Binaural auditory processing in multiple sclerosis subjects. Hearing Research, 68(1), 59–72. [Article] [PubMed]×
Santos, M., Munhoz, M., Peixoto, M., & Silva, C. (2004). High click stimulus repetition rate in the auditory evoked potentials in multiple sclerosis patients with normal MRI. Does it improve diagnosis? Revue de Laryngologie Otologie Rhinologie, 125(3), 151–155. [PubMed]
Santos, M., Munhoz, M., Peixoto, M., & Silva, C. (2004). High click stimulus repetition rate in the auditory evoked potentials in multiple sclerosis patients with normal MRI. Does it improve diagnosis? Revue de Laryngologie Otologie Rhinologie, 125(3), 151–155. [PubMed]×
Figure 1.

Dylan’s Auditory Test Results

Dylan’s Auditory Test Results
Figure 1.

Dylan’s Auditory Test Results

×
2 Comments
July 11, 2016
Karen Ostby
Developmental Delay or Something Else?
Excellent article! M's. ferre went the extra mile to meet the needs of her student. Ashe persisted in covering all areas, using newer instrumentation. She is a tremendous asset for this student, his parents and hisclassroom teacher. This is a story of exceptional skills used to improve the learning of this student.
July 11, 2016
Karen Ostby
Developmental Delay or Something Else?
Excellent article! Contained information I can still process after 46 yrs. Since receiving my MA. It also presented new information, indicating Ms.Ferre went the extra mile to meet the individual needs of her student. She didn't just rely on the usual tests but kept gathering pertinent information. She is an excellent advocate for her students.
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July 2016
Volume 21, Issue 7