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Perinatal thiamine deficiency causes cochlear innervation abnormalities in mice
Stéphane F Maison1, Yanbo Yin2, Leslie D Liberman3
1Department of Otology and Laryngology, Harvard Medical School, Boston MA, USA; Eaton-Peabody Laboratory, Massachusetts Eye & Ear Infirmary, Boston MA, USA; Harvard Program in Speech and Hearing Bioscience and Technology, Boston MA, USA.
Hearing Research
|March 6, 2016
Summary
Neonatal thiamine deficiency damages auditory nerves in mice, specifically impacting inner hair cell synapses. This cochlear pathology leads to auditory neuropathy, even without hair cell loss.
Area of Science:
- Neuroscience
- Otolaryngology
- Developmental Biology
Background:
- Neonatal thiamine deficiency is linked to auditory neuropathy in humans.
- The precise cochlear mechanisms underlying this condition remain unclear.
Purpose of the Study:
- To investigate the cochlear pathology in mice resulting from thiamine deficiency during development.
- To identify specific neural and synaptic abnormalities in the cochlea.
Main Methods:
- Mice were subjected to thiamine-free or low-thiamine diets during fetal or early postnatal development.
- Cochlear function was assessed using otoacoustic emissions and auditory brainstem responses.
- Cochlear histopathology was analyzed via plastic sections and immunostaining for neuronal and synaptic markers.
Main Results:
- Thiamine deprivation did not cause hair cell loss or affect non-sensory cochlear structures.
- Significant reductions in afferent synaptic counts and efferent innervation density were observed in both inner and outer hair cell areas.
- Auditory brainstem response amplitudes were more affected than thresholds, consistent with de-afferentation.
Conclusions:
- Auditory neuropathy in thiamine-deprived mice is associated with neural and synaptic degeneration in the cochlea.
- The primary cause of auditory neuropathy in this model appears to be the loss of inner hair cell synapses.
