Bilateral Profound Acquired Sensorineural Hearing Loss in A Child with Landau Kleffner Syndrome: A Case Report

Background: Landau–Kleffner syndrome (LKS) or acquired epileptic aphasia is a very rare epileptic encephalopathy of childhood presents with regression in receptive and expressive language in a developmentally normal child, with or without clinical seizures. Neither acquired peripheral profound sensorineural hearing loss nor performance after cochlear implantation in Landau–Kleffner syndrome are described in the literature.

Methods: A case report of a child with Landau–Kleffner variant syndrome.

Results: A 5 years old male suffering from bilateral severe-profound hearing loss and a 3 years duration of increasing difficulties in receptive and expressive language was referred to our department. His Neuropediatric specialist suspected of LKS variant syndrome based on clinical presentation and as well as on brain imaging (SPECT) which revealed an alarming hypo-perfusion of the left temporal lobe. CT and MRI of the brain and ears were normal. Pharmacological intervention has led to a clinical improvement and reversal to normal of the pathologic SPECT imaging. Yet, his hearing continues to deteriorate. The child underwent a cochlear implantation. Initial outcome evaluation show normal responses for a post-lingual 5 year old implantee despite the evident neurologic and language events.

Conclusion: A multidisciplinary team assessment is the key for early diagnosis and treatment of hearing loss in complex cases. Clinicians should refer individuals with severe-profound hearing loss for hearing rehabilitation (hearing aids or cochlear implants) as soon as audiologic indication is established. Clinicians should avoid any delays. The damage of long standing deafness is already well proven while potential risks from the interventions, including from cochlear implantation when performed according to commonly accepted standards is low.