NF II and Vestibular Schwannoma

Not too many people were scheduled for clinic this week but I did get to observe an interesting case. A woman who has been diagnosed with neurofibromatosis type II (NF II) came in for a hearing evaluation. There are two types of NF. Type I effects the majority of the body while type II effects mainly the head (which includes the cochleo-vestibular nerve). The patient has already had surgery to remove a tumor in one ear. The surgery resulted in the patient basically having a dead ear. Audiometry revealed that hearing in the opposite ear was not good either (severe-profound loss). The patient wanted to know about cochlear implants, hearing aids, and augmentative communication. Although auditory rehabilitation for her was not likely, Mrs. Haines discussed some computer programs that could possibly help with communication.

Moffat et al. discussed the overall management of patients with NF II. The incidence of NF II is around 1: 40,000. Symptoms tend to become apparent in the second or third decade of life. These include hearing loss, tinnitus, imbalance, and facial paresis. Although hearing loss is usually unilateral, vestibular schwannomas are bilateral. In the study, 23 of the 35 patients underwent surgery. Long-term hearing preservation was not possible in any of these patients. Due to tumor size and poor residual hearing, 11 out of the 15 who were offered surgery as the initial intervention could not have hearing preservation surgery. Although this article does not have many positive outcomes or suggestions, it gives a good overview of the condition and options.

If you haven't seen anything on auditory brainstem implants, I suggest google-ing it. The surgery is geared toward patients with NF II. Crazy new stuff!!!

Moffat, D. et al. (2003). Management strategies in neurofibromatosis type 2. European Archives of Oto-Rhino-Laryngology, 260, 12-18.
http://www.springerlink.com/content/qm8gjt0yw8n2kp2j/fulltext.pdf