How your ears can tell what is hidden in your heart: wild-type transthyretin amyloidosis as potential cause of sensorineural hearing loss inelderly-AmyloDEAFNESS pilot study.

BACKGROUND

Wild-type transthyretin amyloidosis (ATTRwt) is an age-related life-threatening condition. Prognosis is mainly dependent on cardiac involvement. Other organs and tissues may be affected. Their early recognition may increase awareness of physicians and positively affects the prognosis. Presbycusis is another age-related disorder. Whether this disease is associated to ATTRwt amyloidosis is unknown.

METHODS

Sixteen consecutive patients with confirmed diagnosis of ATTRwt amyloidosis at the Mondor Amyloidosis Network, France, underwent otoscopy and audiological tests including pure tone audiometry, speech reception threshold and speech discrimination score.

RESULTS

The mean age was 79 ± 5 years. All were male with an NYHA average of 2.5 ± 0.8. All the patients had sensorineural hearing loss that seemed to preexist to cardiac disorder with greater severity than expected for their age. For speech discrimination test, the mean speech reception threshold was 28 ± 15 dB and the mean speech discrimination score was 68 ± 16 at 40 dB. Ten patients (62.5%) failed to recognize 100% of the words. Compared to age-related expectations according to statistical distribution (ISO), hearing loss included all frequencies and was more severe in patients with ATTRwt amyloidosis.

CONCLUSIONS

These findings suggest that amyloid deposits could infiltrate the various anatomical structures of the inner ear. Description of specific audiologic pattern of ATTRwt amyloidosis might be proposed as a "red flag" and could help for early identification of patients who may be at high risk of ATTRwt amyloidosis as specific treatments are available.