Research Perspective Volume 3, Issue 6 pp 657—662

Besides Huntington's disease, does brain-type creatine kinase play a role in other forms of hearing impairment resulting from a common pathological cause?

Yow-Sien Lin1,2,3, , Chih-Hung Wang4,5,6, , Yijuang Chern1,2,3, ,

  • 1 Molecular Medicine Program, National Yang-Ming University, Taipei, Taiwan;
  • 2 Institute of Neuroscience, National Yang Ming University; Taipei, Taiwan;
  • 3 Division of Neuroscience, Institute of Biomedical Sciences, Academia Sinica, Taipei, Taiwan;
  • 4 Department of Otolaryngology-Head and Neck Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan;
  • 5 Institute of Microbiology and Immunology, National Defense Medical Center, Taipei, Taiwan;
  • 6 Institute of Undersea and Hyperbaric Medicine, National Defense Medical Center, Taipei, Taiwan

Received: June 3, 2011       Accepted: June 14, 2011       Published: June 15, 2011
How to Cite


Hearing impairment following cochlear damage due to noise trauma, ototoxicity caused by aminoglycoside antibiotics, or age-related cochlear degeneration was linked to a common pathogenesis involving the formation of reactive oxygen species (ROS). Cochleae are more vulnerable to oxidative stress than other organs because of the high metabolic demands of their mechanosensory hair cells in response to sound stimulation. We recently showed that patients and mice with Huntington's disease (HD) have hearing impairment and that the dysregulated phosphocreatine (PCr)-creatine kinase (CK) system may account for this auditory dysfunction. Given the importance of noninvasive biomarkers and the easy access of hearing tests, the symptom of hearing loss in HD patients may serve as a useful clinical indicator of disease onset and progression of HD. We also showed that dietary creatine supplementation rescued the impaired PCr-CK system and improved the expression of cochlear brain-type creatine kinase (CKB) in HD mice, thereby restoring their hearing. Because creatine is an antioxidant, we postulated that creatine might enhance expression of CKB by reducing oxidative stress. In addition to HD-related hearing impairment, inferior CKB expression and/or an impaired PCr-CK system may also play an important role in other hearing impairments caused by elevated levels of ROS. Most importantly, dietary supplements may be beneficial to patients with these hearing deficiencies.


CK: creatine kinase; CKB: brain-type creatine kinase; Htt: Huntingtin; HD: Huntington's disease; NII: neuronal intranuclear inclusion; PCr: phosphocreatine; polyQ: polyglutamine; SNHL: sensorineural hearing loss; UHDRS: Unified Huntington's Disease Rating Scale.