Hearing disturbances in hypertrophic cardiomyopathy. Is the sensorineural disorder neurogenic or myogenic?

Miklós Csanády1, Ferenc Tóth, Márta Hogye

  • 12nd Department of Medicine and Cardiology Center, Albert Szent-Györgyi Medical and Pharmaceutical Center, Medical Faculty, University of Szeged, Szeged Korányi fasor 6, H-6720, Hungary. csanady@in2nd.szote.u-szeged.hu

Insights

Patients with hypertrophic cardiomyopathy (HCM) experience more hearing defects, primarily myogenic, than those with dilated cardiomyopathy (DCM) or healthy individuals. These hearing issues in HCM may stem from abnormal sarcomeric proteins affecting inner ear structures.

Area of Science:

  • Cardiology
  • Otolaryngology
  • Genetics

Background:

  • Previous research indicated a high prevalence of hidden hearing defects in hypertrophic cardiomyopathy (HCM) patients.
  • These defects were less common in dilated cardiomyopathy (DCM) and rare in other cardiac conditions or among those on beta-blockers.
  • Initial assessments using brain-stem evoked response audiometry (BAEP) could not definitively distinguish between myogenic and neurogenic origins of hearing loss.

Purpose of the Study:

  • To investigate whether hearing disturbances in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) patients are myogenic or neurogenic.
  • To differentiate the underlying causes of hearing impairment in these patient groups.

Main Methods:

  • Examined inner ear neurogenic function using brain-stem evoked response audiometry (BAEP).
  • Assessed inner ear myogenic function through distortion product otoacoustic emission (DPOAE) technique.
  • Compared findings between HCM patients, DCM patients, and healthy controls.

Main Results:

  • Myogenic abnormalities were identified in 57% of HCM ears and 36% of DCM ears.
  • Neurogenic abnormalities were found in 28% of HCM ears and 21% of DCM ears.
  • Healthy controls exhibited the lowest incidence of both myogenic and neurogenic hearing abnormalities.

Conclusions:

  • Myogenic lesions are more prevalent than neurogenic lesions in hypertrophic cardiomyopathy (HCM) patients.
  • Both myogenic and neurogenic hearing lesions are significantly more common in HCM patients compared to DCM patients and healthy controls.
  • Abnormal sarcomeric proteins in the inner ear's muscular structures are hypothesized as the cause of hearing disorders in HCM, suggesting a non-neurological basis.
Abstract

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