74 Journal of Educational Audiology vol. 15, 2009 Auditory Remediation for a Patient with Landau-Kleffner Syndrome: A Case Study Annette Hurley, PhD Louisiana State University Health Sciences Center, New Orleans, Louisiana Raymond M. Hurley, PhD University of South Florida, Tampa, Florida Landau-Kleffner Syndrome (LKS) is a rare, childhood neurological disorder characterized by a sudden or gradual development of acquired aphasia. This case study offers a unique opportunity to assess the changes in the auditory processing ability of a 12 year old male with LKS after two distinct auditory training programs, Fast ForWord® and Dichotic Interaural Intensity Difference (DIID) training. Improvement in the electrophysiological recordings and the behavioral scores from the Dichotic Digits Test are evidence of the plasticity of the central auditory nervous system and may indicate a viable auditory remediation therapy for persons with LKS. Introduction Landau-Kleffner syndrome (LKS) is a rare childhood neurological disorder characterized by a sudden or gradual development of the inability to understand or express language. LKS is often referred to as acquired epileptic aphasia, acquired aphasia with convulsive disorder, or acquired receptive aphasia (Lees & Urwin, 1991; Paquier, Van Dongen, & Loonen 1992) characterized by an abnormal electroencephalogram (EEG) typifed by abnormal spike activity in the temporal and/or parietal regions (Deonna, 1991). The abnormal EEG activity predominately occurs in the left temporal lobe, but may be present in both temporal lobes (Deonna, 1991) with nocturnal seizures occurring in over 80% of patients with LKS (Patry, Lyagoubi, & Tassinari, 1971). The onset of LKS usually occurs between three and seven years of age, affecting males more often than females (Miller, Campbell, Chapman, & Weismer, 1984). The deterioration in language may be rapid, or may decline over a few months (Miller et al., 1984). Often, because a child with LKS fails to respond to language and environmental sounds, the child is thought to have acquired a hearing loss (Tharpe, Johnson, & Glasscock, 1991). A child with LKS may also be misdiagnosed as having autism or other developmental delays (Tharpe et al., 1991). LKS is known to be heterogeneous with varying symptoms, pathophysiology, degree of impairment, and prognosis. Furthermore, behavioral disturbances, such as aggression, attention, autistic-like behaviors, and withdrawal may be present and related to frustration from the communication breakdown (Tharpe et al., 1991). Previous researchers have reported signifcant auditory discrimination defcits, as well as electrophysiological evidence, suggesting neural coding problems. Specifcally, Vance, Dry, and Rosen (1999) reported on one 14 year old male with auditory discrimination defcits for syllables and words. Baynes, Kegl, Brentari, Kussmaul and Poizner (1998) reported on a 27 year old female with linguistic and non-linguistic auditory discrimination defcits, as well as a defcit in the discrimination of frequency and duration. Stefanatos (1993) has provided electrophysiological evidence suggesting an inability to phase lock to frequency- modulated (FM) steady-state cortical evoked response in a group of children with LKS, but not in a group of children with language impairment. The inability of the auditory system to phase-lock provides objective evidence for the underlying neurophysiological basis of the acoustical analysis of temporal cues, which is important for speech understanding (Kraus & Nicol, 2005). While medical treatment for LKS usually consists of anticonvulsants to treat the seizure activity, there is very little information about the clinical management for the language disorder or acquired (central) auditory processing disorder ([C]APD). Hungerford, Coppens, and Clarke (1998) reported successful implementation of a computer-based language program for one patient with LKS. In addition, Pedro and Leisman (2005) reported signifcant improvement in the auditory, language, and motor skills in a case study of a 14 year old female after completing auditory integration therapy (Interactive Metronome). Treatment of (C)APD generally focuses on three areas: 1) environmental changes to ease communication diffculties, 2) introduction of compensatory skills and strategies for the disorder; and 3) remediation of the auditory defcit. One type of direct remediation of (C)APD is an auditory training program that takes advantage of the brain’s lifelong capacity for plasticity and adaptive reorganization, which may be at least partially reversible through a defcit-specifc training program (Musiek, Chermak, & Weihing, 2007). Brain reorganization is refected in an increase in the number of synapses, increased neural density, and improvement in auditory evoked responses (Elbert, Pantev, Wienbruch, Rockstroh, & Taub, 1995; Merzenich, Schreiner, Jenkins & Wang, 1993; Raconzone, Schreiner, & Merzenich, 1993). Changes in