An electronic survey was sent to US-based patients and caregivers affected by NF2-SWN and non-NF2-SWN who were enrolled in the CTF's NF Registry. Survey topics included demographics, SWN care location, and self-reported clinical care in accordance with published recommendations.

Demographic data was available for 746 patients with NF2-SWN and 204 patients with non-NF2-SWN. Among patients with NF2-SWN, 24% of cases were inherited, 48% reported undergoing genetic testing. In contrast, 45% of non-NF2-SWN patients reported undergoing genetic testing. Despite the complexity of these diseases, only 60% of patients with NF2-SWN and 29% of patients with non-NF2-SWN reported receiving care in a specialized NF clinic. Sixty-four percent of patients with NF2-SWN reported receiving an audiogram in the last 12 months, while 84% reported receiving a brain MRI in the last 12 months and 96% reported receiving a spine MRI in the last 36 months.

The survey revealed that a large proportion of patients living with SWN in the US don't attend a specialized clinic or haven't received recommended genetic testing/counseling. While timely receipt of recommended MRIs of the neuraxis is reported in a high proportion of patients, routine audiograms are performed in about two-thirds of NF2-SWN despite the risk of hearing loss. In the absence of treatment-associated guidelines, there is little current insight into the quality of therapy for these patients. These data serve as a benchmark for organizations that focus on expanding access to expertise for SWN. Development of treatment-associated recommendations would benefit both patients in specialized clinics and elsewhere.