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Temporal bone histopathology in MELAS syndrome.
Ophir Handzel1, Omer J Ungar2, Dan J Lee3
1Cochlear Implant Center, Department of Otolaryngology/Head, Neck & Maxillofacial Surgery Tel-Aviv Sourasky Medical Center, Sackler Faculty of Medicine Tel-Aviv University Israel.
Laryngoscope Investigative Otolaryngology
|March 5, 2020
Summary
Histopathology of temporal bones in MELAS syndrome reveals stria vascularis atrophy, suggesting hearing loss stems from this dysfunction, not hair cell or neuron loss. Findings support cochlear implants for auditory rehabilitation.
Area of Science:
- Otopathology
- Neuroscience
- Mitochondrial Diseases
Background:
- MELAS (myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome is a mitochondrial DNA disorder.
- Previous histopathological studies of MELAS syndrome temporal bones are limited.
Purpose of the Study:
- To describe the histopathology of temporal bones in the oldest MELAS syndrome patient.
- To correlate histopathologic findings with clinical data.
Main Methods:
- Histopathological analysis of temporal bones from the oldest MELAS syndrome donor.
- Correlation of findings with premortem clinical data.
Main Results:
- Severe atrophy of the stria vascularis was observed in the cochleae.
- Organ of Corti and inner hair cells were intact; some outer hair cells were lost.
- Spiral ganglion cell numbers were largely normal.
- Vestibular neuroepithelium showed some degeneration and collapse.
- Cerebral cortex exhibited infarct-like lesions.
Conclusions:
- Hearing loss in MELAS syndrome is likely due to stria vascularis dysfunction, not hair cell or neuron loss.
- Vestibular pathology aligns with in-vivo measurements.
- Findings support auditory rehabilitation, including cochlear implants, for MELAS patients and potentially others with mitochondrial hearing loss.
