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• W3127775867 abstract "Neurofibromatosis type II (NF2) is a rare genetic condition characterized by the slow growth of noncancerous tumors in the nervous system.1-2 Mutation to Schwann cells, which form the myelin sheaths around nerves, is what gives rise to the problem of tumors.Figure 1: The patient's initial testing at 21 years of age. Audiology, case study.Figure 2: (a) Example of a normal ABR from equipment used in this study; (b) the 21-year-old patient's initial ABR testing. Audiology, case study.Figure 3: MRI axial image revealing bilateral vestibular schwanommas and a fourth ventricle tumor. Audiology, case study.Figure 4: The patient's audiogram at 22 years of age conducted after the removal of fourth ventricle tumor. Audiology, case study.Figure 5: The patient's audiogram at 25 years old, following the removal of the left VIII nerve tumor. Audiology, case study.Figure 6: The patient's audiogram at 30 years of age. Audiology, case study.With an incidence estimated to be one in 25,000 to 33,000, NF2 tumors typically arise by 30 years of age, with an average age of diagnosis being 25 years.1-4 The vestibular nerve is most affected by NF2 whereby 90-95 percent of patients develop vestibular schwannomas, unilateral at the onset and eventually becoming binaural.3-4 NF2 can also manifest as acoustic neuromas, meningiomas, and ependymomas. Symptoms of NF2 are dependent on tumor location. However, patients with NF2 often report hearing loss, tinnitus, imbalance, or dizziness as their initial symptoms. NF2 is diagnosed by confirming the presence of vestibular schwannomas or other NF2-related tumors on magnetic resonance imaging (MRI). However, the heterogeneity in NF2 symptoms often leaves patients undiagnosed for an average of seven years after symptom onset.3-5 Audiologists may be the first health professional to assess patients with NF2 given the frequency of vestibular nerve involvement and its associated symptoms. Therefore, the purpose of this case study is to highlight the role of audiologists in facilitating initial NF2 diagnosis by employing audiologic tests that are sensitive to neural dysfunction and properly pursuing additional appropriate “value-added” tests. CASE DETAILS A 21-year-old male presented to an audiologist with complaints of imbalance and hearing difficulties. Pure tone testing revealed a relatively flat mild sensorineural hearing loss in the right ear with normal hearing sensitivity in the left ear (Fig. 1). Speech reception thresholds agreed with the pure tone results. Word recognition (MLV, NU-6 word list) in quiet was good for the right ear and excellent for the left ear. Given the history and preliminary audiological findings, it was recommended that he return the next day for an evaluation of the auditory brainstem response (ABR). However, the patient returned four months later with increased balance issues. At that time, pure tone testing showed about the same results as found previously. ABR testing was performed immediately given the patient's symptoms. The results revealed only Wave I, bilaterally (Fig. 2); all subsequent peaks were absent. Noting the ABR results, the audiologist recommended that the patient seek further medical (ENT) consultation and imaging. The ENT consultation was done on the same day, resulting in the immediate referral for an MRI, which was done two days later. The patient was then diagnosed with NF2 with six tumors identified on MRI: bilateral vestibular schwannomas and four meningiomas (Fig. 3). One meningioma was located within the fourth ventricle and considered potentially fatal due to its size and location. The fourth ventricle meningioma was removed first. An audiogram (Fig. 4) was done following the removal of the fourth ventricle meningioma and prior to removal of the left schwannoma. It showed poorer sensitivity and poorer word recognition for the left ear. Following surgical removal of the left schwannoma, the patient was implanted with a left auditory brainstem implant (ABI) in anticipation of future removal of the right schwannoma. The patient did not continue with the ABI due to his dissatisfaction with the sound quality. He noted that the device sounded like the computer game “Pong.” It was also interesting that static electricity affected the device, causing it to lose its programming. Static electricity is common in New Mexico with its low humidity. Touching a doorknob was enough to disable it. The facility that placed the ABI was contacted and agreed to send software for reprogramming but stated that they always had a crash cart nearby when programming. It was decided that since there was no crash cart in our facility, this would not be pursued. Audiograms representing three and eight years post-op are shown (Figs. 5 and 6). Amplification options were discussed for the right ear, but the patient did not follow-up for amplification. The result of this case study demonstrates the unique role that audiologists have in facilitating the early diagnosis of NF2 and other similar conditions that often go undiagnosed for years after initial symptom onset. Literature supports ABR's sensitivity and usefulness in NF2 while pure tone testing often fails to yield contributory diagnostic value.4,6-8 While audiologists cannot directly diagnose NF2, astute clinical audiologic test selection and execution can herald its timely initial diagnosis, which can be critically important in cases such as these." @default.
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• W3127775867 date "2021-02-01" @default.
• W3127775867 modified "2023-09-27" @default.
• W3127775867 title "ABR Heralds the Initial Diagnosis of Neurofibromatosis Type II" @default.
• W3127775867 doi "https://doi.org/10.1097/01.hj.0000734204.53007.ed" @default.
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