Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab

A case report

Manuela Marsili, Valentina Marzetti, Marta Lucantoni, Giuseppe Lapergola, Marco Gattorno, Francesco Chiarelli, Luciana Breda

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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. Case presentation: 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.

Original languageEnglish
Article number81
JournalItalian Journal of Pediatrics
Volume42
Issue number1
DOIs
Publication statusPublished - Sep 6 2016

Fingerprint

Sensorineural Hearing Loss
Pediatrics
Labyrinth Diseases
Bilateral Hearing Loss
Tinnitus
Uveitis
Dizziness
Ataxia
Therapeutics
Immunosuppressive Agents
Vasculitis
Neurologic Manifestations
Adalimumab
Autoimmune Diseases
Ear
Blood Vessels
Fever
Tumor Necrosis Factor-alpha
Steroids
Skin

Keywords

  • Adalimumab
  • Behçet disease
  • Children
  • Pediatrics
  • Treatment
  • Vasculitis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab : A case report. / Marsili, Manuela; Marzetti, Valentina; Lucantoni, Marta; Lapergola, Giuseppe; Gattorno, Marco; Chiarelli, Francesco; Breda, Luciana.

In: Italian Journal of Pediatrics, Vol. 42, No. 1, 81, 06.09.2016.

Research output: Contribution to journalArticle

Marsili, Manuela ; Marzetti, Valentina ; Lucantoni, Marta ; Lapergola, Giuseppe ; Gattorno, Marco ; Chiarelli, Francesco ; Breda, Luciana. / Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab : A case report. In: Italian Journal of Pediatrics. 2016 ; Vol. 42, No. 1.
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