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"This was shared by an audiologist on a HH list a few years ago where he gave his thoughts on hearing loss & HH.  I have been unable to locate the article which he quotes.  If anyone finds it, please let me know!"


Someone asked about hearing loss.

Simon, A.M., and Bourel, M. Letter to the Editor. Otosclerosis and Vestibular Generation. Archives of Otolaryngology, October 1974, Vol 100.
page 238.

This is my summation of the article.

The authors reported on a couple of earlier articles. They stated that
hearing loss occurred in 14 of 43 (32%) of idiopathic hemochromatosis
patients whereas in a control group only three patients (7%) showed
hearing loss and the difference was statistically significant.

Of the 14 cases of hearing loss, nine were perceptive (which usually means
inner ear and or VII cranial nerve), two were mixed (which usually means
middle ear conductive and perceptive) and three were conductive only.

The authors state that the conductive and mixed losses were possibly
caused by chondrocalcinosis of the ossicle(s) which are the tiny bones in
the middle ear. The authors stated that they may have overestimated the
frequency of hearing loss in H but that the symptom/sign does belong in
the clinical picture.

One of their references was a histologic case study that showed macula and
saccule otolithic (inner ear balance mechanism) degeneration.

>From a practical point of view this information is important to your
otolaryngologist and or audiologist so that they know what to search for
if you are having hearing problems. Perceptive or neurosensory hearing
loss is not generally reversible unless related to hypothyroid in which
cas it is reversible about 25% of the time. However, conductive and mixed
hearing loss is often reversible or partly reversible with surgery. If
you do have hearing problems and HH believe me when I tell you that the
majority of practitioners out there will not know there is a link between
the two and in fact I think the evidence is still sketchy. But
nonetheless, if you do have the problem, you may want to give your
practitioner the reference listed above.

Most importantly, if you had documented hearing loss and it improved after
phelbotomy (which I haven't heard of yet), then it would be worth writing
up a case study because you would be adding to the body of knowledge in
the literature. However, it is unlikely that the types of hearing loss
outlined in the articles would improve. The only type that I would expect
to improve would be a sensorineural loss that was related to
hypothyroidism and even then only 25% of the time.

Richard Primeau
Audiologist