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Bilateral objective tinnitus in an infant with tuberous sclerosis
Masafumi Ohki1, Shigeru Kikuchi
1Department of Otolaryngology, Saitama Medical Center, Kawagoe-shi, Saitama, Japan. m-ohki@umin.ac.jp
International Journal of Pediatric Otorhinolaryngology
|November 21, 2012
Summary
This study reports objective tinnitus, specifically audible spontaneous otoacoustic emissions, in a patient with tuberous sclerosis. This finding suggests potential auditory system dysfunction in tuberous sclerosis patients.
Area of Science:
- Neuroscience
- Otolaryngology
- Genetics
Background:
- Tuberous sclerosis is a genetic disorder affecting multiple organs.
- Auditory system involvement in tuberous sclerosis is not well-documented.
- Objective tinnitus is a rare condition where the sound originates within the patient.
Observation:
- A patient with tuberous sclerosis presented with loud, continuous, high-pitched objective tinnitus.
- The tinnitus was identified as audible spontaneous otoacoustic emission (SOAE).
- SOAEs were measured at high frequencies (7757.8 and 6257.8 Hz).
Findings:
- This is the first reported case of objective tinnitus as SOAE in tuberous sclerosis.
- Abnormal myelination and dysfunctional neural elements associated with tuberous sclerosis are potential causes.
- Disturbances in outer hair cells or medial olivocochlear (MOC) efferent fibers are implicated as the source of SOAE.
Implications:
- Suggests potential auditory system dysfunction in tuberous sclerosis.
- Highlights the need for audiological evaluation in tuberous sclerosis patients.
- Opens avenues for research into the mechanisms of tinnitus in neurocutaneous disorders.
