Evidence-Based Guideline: Evaluation, Diagnosis, and Management of Facioscapulohumeral Dystrophy


Tawil, R., Kissel, J. T., et al. (2014).

Minneapolis (MN): American Academy of Neurology; American Association of Neuromuscular & Electrodiagnostic Medicine, 1-44.

This guideline provides recommendations for the evaluation, diagnosis, and treatment of facioscapulohumeral muscular dystrophy (FSHD). Childhood-onset FSHD with genetic abnormalities may result in significant, progressive hearing loss that requires monitoring and amplification. The target population of the guideline includes individuals across the lifespan. This summary highlights recommendations regarding the importance of screening young children for hearing loss within the scope of audiology.

American Academy of Neurology; American Association of Neuromuscular & Electrodiagnostic Medicine

FSH Society; Muscular Dystrophy Association

<p>This guideline was reviewed with the following supporting document:</p> <ul> <li>Tawil, R., Kissel, J.T., et al. (2015). Evidence-Based Guideline Summary: Evaluation, Diagnosis, and Management of Facioscapulohumeral Muscular Dystrophy: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the Practice Issues Review Panel of the American Association of Neuromuscular &amp; Electrodiagnostic Medicine. <i>Neurology, 85</i>(4): 357-364. doi: 10.1212/WNL.0000000000001783</li> </ul>




<div>"Clinicians should screen all young children with FSHD [facioscapulohumeral dystrophy] at diagnosis and yearly thereafter until these children start school, as hearing loss may not be present at diagnosis and can be progressive (Level B Evidence)" (p. 17).</div>