Dear Readers:
I am out on vacation this week but will be back with another all new post for Hearing International on April 1, 2015. Here’s a post from a previous week that was popular.
It is well known that Mozart had an ear for music. (Enjoy The Best of Mozart at YouTube.) What is not so well known is that he also had a condition of the pinna that has, over the centuries, become known by the eponym “Mozart Ear”.
Mozart’s Ear Left, Normal Ear Right
According to Yamashita et al. (2011), Mozart Ear is an auricular deformity characterized by a bulging appearance of the anterosuperior portion of the auricle, a convexly protruded cavum conchae, and a slit-like narrowing of the orifice of the external auditory meatus. Some have described it as a fusion of the cura of the anti-helix with the cura of the helix. It is said to be uncommon, and because no one has yet fully described either the disease or the treatment, there is no unified concept of Mozart ear. The anomaly, also known as Wildermuth Ear for Hermann A. Wildermuth (1852–1907), the German neurologist who identified the defect, is said to have affected Wolfgang Amedeus Mozart and his son Franz. Wildermuth Ear describes a congenital defect characterized by a backwards-oriented helix (the pinna’s curved, cartilage border), which deforms the ear by protruding the ante-helix (inner ridge of the pinna).
There is a lot of speculation as to how Mozart died at age 35. Direct medical evidence? None. Autopsy? Not performed. Medical records? Nowhere to be found. Corpse? Disappeared………So no one knows what actually happened to Mozart. It is possible that his death had nothing to do with poisoning, or the other 118 other probable causes of death offered by Karhausen (1998). Since that time, however, there have been some indications from imaging and other findings that offer a connection between the Mozart ear malformation and renal urinary disease.
Wang et al. (2001), in a review of ear anomalies and renal ultra sounds, found that among those with this type of ear malformation there was an increased frequency of clinically significant structural renal anomalies compared with the general population. Izzedine et al. (2004) suggest that the embryogenesis of the association of 
renal and auricular anomalies is unclear. The combination of ear and kidney anomalies in the early stages of development can be explained on the assumption that mesodermal induction (transcription factor, gene expression) is responsible for normal differentiation of both organs.
While other suggested causes of Mozart’s demise are possible, Karhausen concluded “the risk of having a congenital anomaly of the urinary tract was a hundred times higher with an external ear anomaly [such as Mozart’s ear] than in the general population.” Unless significant new information is miraculously discovered, the exact cause of Mozart’s death will never be known, but renal/urinary tract disease is a distinct possibility.
References:
Wakin, D. (2010). After Mozart’s death, an endless coda. New York times. Retrieved December 16, 2014: https://www.nytimes.com/2010/08/25/arts/music/25death.html?_r=0
Stevenson, R. (2006). Human Malformations and Related Anomalies. Retrieved December 16, 2014: https://books.google.com/books?id=_ssqN2gaDoUC&pg=PA349&lpg=PA349&dq=auditory+effects+mozart+ear&source=bl&ots=TykXAbhrnK&sig=FKIvQ1NJv1S1LedJu0AaIDbOZsY&hl=en&sa=X&ei=uzuQVLXnKYSsyATphYCoDw&ved=0CFcQ6AEwCA#v=onepage&q=auditory%20effects%20mozart%20ear&f=false
Yamashita K., Yotsuyanagi T., Saito T., Isogai N., Mori H. & Itani Y. (2011). Mozart Ear: Diagnosis, treatment and literature review. Ann Plast Surg. 2011 Nov;67(5):547-50. Retrieved December 16, 2014: https://www.ncbi.nlm.nih.gov/pubmed/21587051
Wang, R., Earl, D., Ruder, R., & Graham, J. (2001). Syndromic anomalies and renal ultrasounds. Pediatrics. 2001 Aug;108(2). Retrieved December 16, 2014: https://www.ncbi.nlm.nih.gov/pubmed/11483842
Wang RY1, Earl DL, Ruder RO, Graham JM Jr.
Images:
Mozart, Wolfgang Amadeus, 1756-1791. Mein Ohr [und] ein gewöhnliches Ohr [My ear and an ordinary ear] : drawing, [n.p., n.d.] Water-color drawing; [n.p, n.d] 1 drawing : watercolor on paper: Retrieved December 16, 2014: https://kottke.org/13/07/mozart-had-weird-ears
Ruehlow, S (2009). Mozart Tombstone. Cemetery Explorers. Retrieved December 16, 2014: https://cemeteryexplorers.blogspot.com/2009/12/tombstone-tales-tomb-of-mozart.html
Videos:
S. Soundiva Orchestra (2013). Eine kleine nachtmusik: 1st movement. Wolfgang Amaedeus Mozart. Halidon. Retrieved December 16,2014: www.youtube.com/watch?v=Rb0UmrCXxVA
