Taste perception and sensory sensitivity: Relationship to feeding problems in boys with Barth Syndrome.

Background: Feeding problems are common in boys with Barth syndrome and may contribute to the population's propensity for growth delay and muscle weakness. The purpose of this study was to quantify and describe these feeding issues and examine altered taste perception and sensory sensitivity as contributing factors.

Methodology: A cross-sectional, two-group comparison design was used to examine feeding preferences and behaviors, chemical taste perception, and sensory sensitivities in fifty boys with (n=24) and without (n=26) Barth ages 4-17 years. Taste perception was measured using chemical test strips saturated with phenylthiocarbamide (PTC) and sodium benzoate (NaB). Feeding problems were documented by parents using a Food Inventory, while sensory sensitivities were recorded using a Short Sensory Profile.

Results: Boys with Barth differed significantly from typical peers with regards to problem feeding behaviors. For boys with Barth, food refusal and food selectivity were identified as being present in 50% the sample, while 70% of had identified problems related to gagging or swallowing foods. About half of all Barth families noted that their child's eating habits did not match the family's and that separate meals were often prepared. As demonstrated in previous research, about 50% of boys with Barth demonstrated probable or definite differences in taste/smell sensitivity, which was significantly higher than controls. On tests of chemical taste perception, boys with Barth were significantly more likely to be supertasters to PTC and non-tasters to NaB. Taster-status did not directly relate to the presence of feeding problems, however, taste/smell sensitivity did significantly relate to food selectivity by type and texture.

Conclusions: Results indicate feeding problems in at least 50-70% of boys with Barth syndrome, and suggest that behaviors are often present before 6 months of age. Differences in taste perception may influence dietary choices in boys with Barth, particularly their craving of salty foods. Taste/smell sensitivity also appears to influence food selectivity, and therefore may be important to consider in this population, particularly in light of dietary influences on cardiac function, energy consumption, and overall growth.