Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab: a case report.
BACKGROUND
Autoimmune sensorineural hearing loss, also known as autoimmune inner ear disease (AIED) is a rare clinical entity characterized by progressive and bilateral sensorineural hearing loss often accompanied by vestibular symptoms. Diagnosis is essential as a consistent number of patients show a positive response to steroids alone or in association with other immunosuppressive drugs. AIED is defined as primary when the disease is limited to the ear, whereas in up to a third of cases it is associated to other systemic autoimmune diseases such as Behçet disease (BD). BD is a rare multisystem vasculitis characterized by recurrent oral and genital aphtosis, uveitis, skin lesions, neurological and vascular manifestations. Clinical presentation is variable thus making the diagnosis difficult in many instances. The choice of therapy is also limited by the scarceness of high-quality therapy studies.


METHODS
We present a 15-year-old-boy with six months of history of fever, dizziness, tinnitus and ataxia. He had a final diagnosis of AIED associated to BD and was successfully treated with the anti-tumor necrosis factor (TNF)-α adalimumab.


CONCLUSIONS
This case report points out to the diagnostic and therapeutic challenges of BD especially when unusual symptoms are the prominent manifestations of the disease. It also suggests that adalimumab is a good therapeutic option in children with BD and audiovestibular symptoms.
Department of Pediatrics, University of Chieti, Via dei Vestini 5, Chieti, 66100, Italy. manuela.marsili@gmail.com.
Manuela
M
Marsili
Department of Pediatrics, University of Chieti, Via dei Vestini 5, Chieti, 66100, Italy.
Valentina
V
Marzetti
Department of Pediatrics, University of Chieti, Via dei Vestini 5, Chieti, 66100, Italy.
Marta
M
Lucantoni
Department of Pediatrics, University of Chieti, Via dei Vestini 5, Chieti, 66100, Italy.
Giuseppe
G
Lapergola
UO Pediatria II, G. Gaslini Scientific Institute, Genova, Italy.
Marco
M
Gattorno
Department of Pediatrics, University of Chieti, Via dei Vestini 5, Chieti, 66100, Italy.
Francesco
F
Chiarelli
Department of Pediatrics, University of Chieti, Via dei Vestini 5, Chieti, 66100, Italy.
Luciana
L
Breda
D000068879:Adalimumab / Q000627:therapeutic use*; D000293:Adolescent; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000209:etiology*; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy*; D003937:Diagnosis, Differential; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis* / Q000209:etiology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male
D000893:Anti-Inflammatory Agents; D000068879:Adalimumab
Adalimumab; Behçet disease; Children; Pediatrics; Treatment; Vasculitis