• Publications
  • Influence
Position-dependent patterning of spontaneous action potentials in immature cochlear inner hair cells
Spontaneous action potential activity is crucial for mammalian sensory system development. In the auditory system, patterned firing activity has been observed in immature spiral ganglion andExpand
  • 121
  • 17
Nuclear Localization of Ataxin-3 Is Required for the Manifestation of Symptoms in SCA3: In Vivo Evidence
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly inherited neurodegenerative disorder caused by the expansion of a CAG repeat in the MJD1 gene resulting in an expanded polyglutamineExpand
  • 161
  • 16
Resting Potential and Submembrane Calcium Concentration of Inner Hair Cells in the Isolated Mouse Cochlea Are Set by KCNQ-Type Potassium Channels
Cochlear inner hair cells (IHCs) transduce sound-induced vibrations into a receptor potential (RP) that controls afferent synaptic activity and, consequently, frequency and timing of actionExpand
  • 122
  • 11
Advances in the neurobiology of hearing disorders: Recent developments regarding the basis of tinnitus and hyperacusis
The prevalence of hearing problems in the Western world has, due to aging of the population, doubled over the past 30 years. Thereby, noise-induced hearing loss is an important factor that worsensExpand
  • 156
  • 10
Functional significance of channels and transporters expressed in the inner ear and kidney.
A number of ion channels and transporters are expressed in both the inner ear and kidney. In the inner ear, K(+) cycling and endolymphatic K(+), Na(+), Ca(2+), and pH homeostasis are critical forExpand
  • 187
  • 10
Scaffold protein harmonin (USH1C) provides molecular links between Usher syndrome type 1 and type 2.
Usher syndrome (USH) is the most frequent cause of combined deaf-blindness in man. USH is clinically and genetically heterogeneous with at least 11 chromosomal loci assigned to the three USH typesExpand
  • 140
  • 10
Thyroid hormone is a critical determinant for the regulation of the cochlear motor protein prestin
The most impressive property of outer hair cells (OHCs) is their ability to change their length at high acoustic frequencies, thus providing the exquisite sensitivity and frequency-resolving capacityExpand
  • 111
  • 10
A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy.
Autosomal dominant optic atrophy (adOA) is a juvenile onset, progressive ocular disorder characterized by bilateral loss of vision, central visual field defects, colour vision disturbances, and opticExpand
  • 205
  • 9
The Reduced Cochlear Output and the Failure to Adapt the Central Auditory Response Causes Tinnitus in Noise Exposed Rats
Tinnitus is proposed to be caused by decreased central input from the cochlea, followed by increased spontaneous and evoked subcortical activity that is interpreted as compensation for increasedExpand
  • 98
  • 9
Tonotopic Variation in the Calcium Dependence of Neurotransmitter Release and Vesicle Pool Replenishment at Mammalian Auditory Ribbon Synapses
The mammalian cochlea is specialized to recognize and process complex auditory signals with remarkable acuity and temporal precision over a wide frequency range. The quality of the informationExpand
  • 102
  • 9