FaCD Online Syndrome Fact Sheet

Last updated: 03 Jul 2008

Name: Neuroendocrine Salivary Gland Cancer, Hearing Loss and Enamel Hypoplasia, Familial

Mode of Inheritance: XLD?/ AD?

Tumor features

neuroendocrine salivary gland tumor

Tumor features (possible)

vestibular schwannoma

Non-tumor features

enamel hypoplasia (amelogenesis imperfecta)
hearing loss, neurosensory


Michaels et al.[1] reported a 3-generation family with low-grade (metastatic) neuroendocrine carcinomas of the salivary glands (submandibular glands, minor salivary-type glands of nasal and maxillary sinuses, ages at diagnosis 10-36 years), severe sensorineural hearing loss (onset 7 and 25 years), attacks of vertigo, enamel hypoplasia (amelogenesis imperfecta) and in 1 male an unilateral vestibular tumor, possibly a schwannoma.


[1] Michaels L, Lee K, Manuja SL, Soucek SO. Family with low-grade neuroendocrine carcinoma of salivary glands, severe sensorineural hearing loss, and enamel hypoplasia. Am J Med Genet 83[3], 183-186. 1999.