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Mondini cochlea in Pendred's syndrome. A histological study
Acta Oto-Laryngologica
|September 1, 1986
Summary
Histological examination of temporal bones confirms the characteristic Mondini cochlea in Pendred syndrome. This inner ear malformation aligns with Mondini
Area of Science:
- Otolaryngology
- Genetics
- Pathology
Background:
- Pendred syndrome is a common cause of inherited hearing loss.
- Previous histological descriptions of inner ear malformations in Pendred syndrome were limited.
Observation:
- This study examined six temporal bones from five patients with confirmed Pendred syndrome.
- Histological analysis revealed the characteristic Mondini cochlea in all specimens.
Findings:
- The inner ear malformation in Pendred syndrome is consistent with Mondini's original description.
- This finding differentiates it from other syndromes with Mondini-like cochlear malformations.
Implications:
- Provides a clearer histological definition of inner ear malformations in Pendred syndrome.
- Aids in the differential diagnosis of congenital hearing loss and cochlear malformations.
Related Concept Videos
01:22Hair Cells
Hair cells are the sensory receptors of the auditory system—they transduce mechanical sound waves into electrical energy that the nervous system can understand. Hair cells are located in the organ of Corti within the cochlea of the inner ear, between the basilar and tectorial membranes. The actual sensory receptors are called inner hair cells. The outer hair cells serve other functions, such as sound amplification in the cochlea, and are not discussed in detail here.
01:13The Cochlea
The cochlea is a coiled structure in the inner ear that contains hair cells—the sensory receptors of the auditory system. Sound waves are transmitted to the cochlea by small bones attached to the eardrum called the ossicles, which vibrate the oval window that leads to the inner ear. This causes fluid in the chambers of the cochlea to move, vibrating the basilar membrane.