title: Unique transgenic animal model for hereditary hearing loss.
abstract: OBJECTIVE
  This study capitalizes on the unique molecular and developmental similarities between the auditory organs of Drosophila and mammals, to investigate genes implicated in human syndromic and nonsyndromic hearing loss in a genetically tractable experimental animal model, the fruit fly Drosophila.
  
  
  METHODS
  The Drosophila counterparts of 3 human deafness genes (DIAPH1/DFNA1, ESPN/DFNB36, and TMHS/DF-NB67) were identified by sequence similarity. An electrophysiological assay was used to record sound-evoked potentials in response to an acoustic stimulus, the Drosophila courtship song.
  
  
  RESULTS
  Flies with mutations affecting the diaphanous,forked, and CG12026/TMHS genes displayed significant reductions in the amplitude of sound-evoked potentials compared to wild-type flies (p < 0.05 to p < 0.005). The mean responses were reduced from approximately 500 to 600 microV in wild-type flies to approximately 100 to 300 microV in most mutant flies.
  
  
  CONCLUSIONS
  The identification of significant auditory dysfunction in Drosophila orthologs of human deafness genes will facilitate exploration of the molecular biochemistry of auditory mechanosensation. This may eventually allow for novel diagnostic and therapeutic approaches to human hereditary hearing loss.
authors.affiliation: Department of Otolaryngology, New York Eye and Ear Infirmary, New York, New York, USA.
authors.forename: Maura
authors.initials: M
authors.lastname: Cosetti
authors.forename: David
authors.initials: D
authors.lastname: Culang
authors.forename: Sumankrishna
authors.initials: S
authors.lastname: Kotla
authors.forename: Peter
authors.initials: P
authors.lastname: O'Brien
authors.forename: Daniel F
authors.initials: DF
authors.lastname: Eberl
authors.forename: Frances
authors.initials: F
authors.lastname: Hannan
mesh_terms: D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics*; D000818:Animals; D004247:DNA / Q000235:genetics*; D004252:DNA Mutational Analysis; D003638:Deafness; D004195:Disease Models, Animal; D004330:Drosophila; D005072:Evoked Potentials, Auditory; D005260:Female; D000080865:Formins; D020022:Genetic Predisposition to Disease; D034381:Hearing Loss / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D008565:Membrane Proteins / Q000235:genetics*; D008840:Microfilament Proteins / Q000235:genetics*; D009154:Mutation
chemical_list: D048868:Adaptor Proteins, Signal Transducing; C108962:DIAPH1 protein, human; C493274:ESPN protein, human; D000080865:Formins; C515721:LHFPL5 protein, human; D008565:Membrane Proteins; D008840:Microfilament Proteins; D004247:DNA