02000caa a2200241   4500008004100000022001400041100002000055245008600075260000900161270008000170270002200250300001100272490001400283520118000297653006001477700001501537700001801552700003001570773005701600856006901657942001501726999001701741210629s9999    xx            000 0 und d  a2582-4961  aPradeep Yuvaraj 0aResolving Auditory Neuropathy Spectrum Disorder in a Case with Leigh’s Disease   c2016  aAll India Institute of Speech and Hearing, Manasagangothri, Mysore, 570 006  mrmjay16@gmail.com  a 14-18  vVolume 35  aLeigh's Disease is a rare inherited neuro-metabolic disorder that aects the central nervous system in infants and children. The disorder is characterized by progressive neurologic deterioration. Patients with Leigh syndrome show inconsistent or absent ABR and abnormal interpeak latencies. Auditory neuropathy spectrum disorder has also been reported. Master PM, 2.8 years old, 2nd born to non-consanguineous parents was brought to our department with the complaints of inconsistent response to auditory stimuli and regression of developmental milestones including that of speech-language. MRI brain showed bilateral T2 FLAIR hyper intense signal changes in several sub-cortical regions of the brain including basal ganglia. ABR results showed ringing cochlear microphonics up to 60dB nHL in both the ears. ABR showed presence of 5th peak up to 70dB nHL in the right ear and 60dB nHL in the left ear. Oto-acoustic emissions were present in both the ears. Based on the results of audiological evaluation and improvement shown by PM in speech-language  following neurological intervention, it is speculated that PM may have had a transient form of ANSD during his development.   aLeigh’s disease, Auditory neuropathy spectrum disorde  aAlphy Sabu  aAnjana Thomas  aJayaram Mannarukrishnaiah  aJournal of All India Institute of Speech and Hearing  uhttp://192.168.102.7/jaiish/index.php/aiish/article/view/919/595  cFJASyFJAS  c17963d17963