Naxos disease (also known as "diffuse non-epidermolytic palmoplantar keratoderma with woolly hair and cardiomyopathy" or "diffuse palmoplantar keratoderma with woolly hair and arrhythmogenic right ventricular cardiomyopathy", first described on the island of Naxos by Nikos Protonotarios) is a cutaneous condition characterized by a palmoplantar keratoderma. The prevalence of the syndrome is up to 1 in every 1000 people in the Greek islands.It has been associated with mutations in the genes encoding the proteins desmoplakin, plakoglobin, desmocollin-2, and SRC-interacting protein (SIP). Naxos disease has the same cutaneous phenotype as the Carvajal syndrome. Symptoms
Between 80 and 99% of those with Naxos disease will display some of the following symptoms:

Disease of the heart muscle
Thickening of palms and soles
Sudden increased heart rate
Dizzy spells
Kinked hair

See also
Olmsted syndrome
List of cutaneous conditions
List of conditions caused by problems with junctional proteins

References


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However, in some cases, the perforation can last several years and will be unable to heal naturally. For patients with persistent perforation, surgery is usually undertaken to close the perforation. The objective of the surgery is to provide a platform of sort to support the regrowth and healing of the tympanic membrane in the two weeks post surgery period. There are two ways of doing the surgery:

Traditional tympanoplasty, usually using the microscope and performed through a 10 cm incision behind the ear lobe. This technique was introduced by Wullstien and Zollner and popularized by the Jim Sheehy at the House Ear Institute. Endoscopic tympanoplasty, usually using the endoscope through the ear canal without the need for incision. This technique was introduced and popularized by Professor Tarabichi of TSESI: Tarabichi Stammberger Ear and Sinus Institute.The success of surgery is variable based on the cause of  perforation and the technique being used. Predictors of success include traumatic perforation, dry ear, and central perforations. Predictors of failure includes young age and poor eustachian tube function. The use of minimally invasive endoscopic technique does not reduce the chance of successful outcome. Hearing is usually recovered fully, but chronic infection over a long period may lead to permanent hearing loss. Those with more severe ruptures may need to wear an ear plug to prevent water contact with the ear drum. References


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