The Facial Nerve (CN VII).-Chorda tympani branch is responsible for the taste innervation.15 Failed embryogenesis during this time period contributes to aglossia. Musculature of tongue, innervated by the Hypoglossal Nerve (CN XII), except for the palatoglossus (which is supplied by the Vagus nerve) is formed by the Occipital somites.
The Occipital somites migrate normally, but because of agenesis of first arch, they are unable to form musculature in cases of aglossia.13,14
The Portuguese mother mentioned in the de Jussieu report, that she used to enlarge the opening of the nursing bottle and milk was poured directly into the infant's throat.3 The earliest association of tongue and limb anomalies, occurring in conjunction, was reported by Kettner in 1907.3 A true case of Aglossia-Adactylia was first documented by Rosenthal in 1932.3 Isolated Congenital Aglossia was reported by Kumar et al in a four-year old Indian female child.7 Rasool et al also reported a similar finding in a six-year old Indian male child.12 The earliest case of Isolated Aglossia in an adult was reported in 1949 in Washington; a 22-year old Chinese man reported with the complaint of pain in lower anterior teeth.
Gradual hypertrophy of the muscles of floor of mouth is a common finding in most cases of aglossia, especially of the Mylohyoid muscle, possibly due to constant stimulation during swallowing.
Taste is a subjective sensation and taste perceptions in aglossia patients have been documented to be intact.18 This can be attributed to the fact that taste buds are present not only on the tongue but also on the floor of mouth, epiglottis, soft palate and mucous membrane projection which is actually a remnant of embryonic buccopharyngeal membrane.
Possible treatments of aglossia sequelae have been discussed in literature.
On January 14th 2013, two famous cases of the very rare and unusual Isolated Congenital Aglossia were presented in a symposium held in Brazil.
Oromandiabular limb hypogenesis syndrome: A case of aglossia with an intra-oral band.
Conductive deafness in aglossia. J Laryngol Otal 1996; 110: 1057-59.
Congenital aglossia with Situs inversus totalis - a case report.
Thyroid dysfunction in a patient with aglossia, Am J Med Genet 2003; 122 (3): 274-277.
Isolated aglossia in a six year old child presenting with impaired speech: A case report.