Our data suggest that some patients with refractory headaches and endonasal contact areas benefit from surgery, thereby supporting the existence of a connection between the two. Even though it is clear that surgery should be considered only if all other treatments have failed, a success rate of 65% over almost 10 years justifies evaluation of this option. Preoperative patient selection remains crucial and warrants further investigation.

The evidence in the literature regarding an appro-priate evaluation is mostly of low quality and difficult to assess owing to high heterogeneity. Nevertheless, imaging, genetic testing, neuropaediatric and ophthalmological evaluations, electrocardiograms and cytomegalovirus analysis have been identified as examinations to be included in the assessment of children with congenital SNHL. There is a need for international consensus on the various issues of such an evaluation, such as choice of investigations and diagnostic criteria.