Journal of Clinical Case Reports

ISSN: 2165-7920

Open Access

Christine Vella

Department of Surgery, Otorhinolaryngology, Mater Dei Hospital, Malta

  • Case Report   
    A Rare Case of Bilateral Duane Syndrome Type I with Inner Ear Developmental Arrest
    Author(s): Christine Vella*, John Cutajar and Edith Vassallo

    Background: Duane Syndrome is a congenital cranial dysinnervation disorder that primarily restricts eye movements. The majority of patients have isolated Duane syndrome with no other congenital abnormalities, however in certain cases, there are non-ocular symptoms which do not fit aspecific syndrome. In this case we describe bilateral Duane syndrome type 1(limited eye abduction) with findings of developmental arrest in the inner ear and cochlear aplasia which to our knowledge has not been reported yet. Case presentation: This case reports a 3 year old boy who presented with watery eyes during feeding and speech delay. He had a right sided sensorineural hearing loss, with ABR being inconclusive and ASSR showed severe hearing loss. CT mastoids and MRI were performed and a diagnosis of cochlear aplasia with inner ear developme.. Read More»
    DOI: 10.37421/2165-7920.2022.12.1544

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